Cystic fibrosis is a genetic disease that causes mucous-secreting glands to produce abnormal secretions, resulting in improper lung tissue lubrication and subsequent damage.
The mucous in patients with cystic fibrosis tends to be very thick, making it impossible for the lungs to be cleared properly of bacteria. This invites multiple infections (bronchitis, Pneumonia, bronchiectasis), atelectasis (collapse of lung tissue), and lung scarring.
Cystic fibrosis is a very serious illness in which only about half of those affected live past 20 years of age. It is the most commonly inherited disease leading to death among the white population of the U.S.
Failure to pass meconium within first 24-48 hours of life (abdominal distention, emesis)
Frequent, bulky, greasy stools and poor weight gain due to pancreatic problems
Constant lung infections
Infertility
Cough
Fatigue on exercise
Decreased muscle mass
Poor growth
Delayed maturation
Epigastric pain
Pallor due to hemolytic anemia
Bleeding problems
Night blindness
Delayed sexual development
Salty taste when you kiss the child
Examination:
Clubbed fingers
Chest is widened front-to-back
Lung exam has crackles
Nasal polyps
Tenderness in right upper abdomen
Laboratory findings:
Arterial blood gas shows decreased oxygenation
Pulmonary function test shows decreased lung function.
