CF

Question 1

In cystic fibrosis there’s a lack of pancreatic pancreatic enzymes which increases the stickiness of mucus in the lungs in G.I. tract and also the pancreatic ducts and the bile ducts. It also causes a decrease in chloride ion transport which causes excessive salt loss. This can be diagnosed by _______.

Answer 1

chloride sweat test

Question 2

There is pulmonary involvement which occurs 90% in patients surviving the neonatal. ______ is the principal cause of death.

Answer 2

End stage lung disease

Question 3

Reduced fluid in the epithelial lining can also cause intestinal ileus, gallstones, decreased bicarbonate production, for digestion by the pancreas, and viscous bile in the liver causing bile duct plugs. This increased stickiness blocks sweat glands and causes pancreatic ________. This patient needs to replace pancreatic enzymes on food.

Answer 3

fibrosis

Question 4

_____ and ______ should be given with applesauce and NOT PROTEIN. This is the pancreatic enzyme replacement. This is also tested for on the newborn screening. _______ is the first sign that your newborn has cystic fibrosis.

Answer 4

Creon and Viokase

Meconium ileus

Question 5

Acid suppression therapy using proton pump inhibitor’s.
Chest physiotherapy to loosens secretions.
Enemas to excrete sticky bowel movements. Immunizations to decrease respiratory infections.
Chest vest physiotherapy.
These are all treatments for ________.

Answer 5

cystic fibrosis

Question 6

This is the most lethal inherited disease and white people. Cystic fibrosis is an autosomal recessive disorder and most carries of the gene are asymptomatic. This is caused by defect in the gene for ______. Ask mom about family history Asea. Involve the lungs and exocrine glands.

Answer 6

cystic fibrosis transmembrane conductance regulator