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CF Quiz

Cystic Fibrosis mini quiz

What is Cystic Fibrosis? Life-threatening genetic disease that causes mucus to build up and clog some of the organs in the body, paticulary the lungs and pancreas.
When mucus clogs lungs -makes breathing difficult -causes bacteria to get trapped in airway -causes inflammation and infections -leading to lung damage
When mucus clogs the digestive tract and pancreas The mucus stops digestive enzymes from getting to the intestines.
How do people with CF digest their food? By taking replacement enzymes with meals and snacks- Helps them digest food to get proper nutrition
Etiology of CF Autosomal Recessive Genetic Disease
Who gets CF? -Approximately 30,000 children and adults in the US have CF -One in every 31 Americans are carriers of the defective CF gene -CF is most common in Caucasians, but it can affect all races
S/S of CF -Very salty tasting skin -Persistent cough, at times with phlegm -Frequent lung infections -Wheezing or SOB -Poor growth/weight gain in spite of good appitite -Frequent bulky stools or difficulty in bowel movements
How does the type of CF gene mutation a person has affect them? Affects the severity of CF symptoms
How many different CF mutations have been identified? More than 1,400 different mutations
How is CF diagnosed? Sweat test - most common
What is a sweat test? -An electrode is placed on the skin (usually the arm) to stimulate the sweat gland -Sweat is collected and the amount of chloride is measured -High chloride level means person has CF
What is Chloride? a component of salt in the sweat
What is the function of a healthy CF gene? Makes a protein called CFTR
What is CFTR? Cystic Fibrosis conductance Transmembrane Regulator
Where is CFTR found? in the cells that line various organs, like the lungs and pancreas
What is the function of CFTR? Controls the movement of ions, like chloride and sodium in and out to these cells.
What happens when the CFTR protein is defective? -the salt balance in the body is disturbed -The mucus in the respiratory tract becomes thick(because there is too little salt and water on the outside of the cells) -Thick mucus clogs the airway which leads to infections that damage lungs.
Tx for CF -take capsules containing pancreatic enzymes with every meal and most snacks. -Take multi-vitamins -Use a mucolytic four or more times a day
What is a Mucolytic? medication that breaks down mucus
Prognosis for CF Medial survival age for CF in the US is 38 years.
Two factors that affect a person with CF chance of survival. -Severity of disease -Time of diagnosis
Created by: owossopatho