Cystic Fibrosis

Question 1

What is CF?

Answer 1

• An inherited autosomal recessive disease

- Result of gene mutation on chromosome 7

Question 2

What is Chromosome 7 responsible for?

Answer 2

• For diffusion of ions across cell membranes
• It controls the flow of water and chloride between membranes
• Sticky mucus (thick and stagnant) builds up outside the cell, not letting anything pass between > nice place for bacteria and infections > results in inflammation and scarring

Question 3

CF affects which systems?

Answer 3

Respiratory - natural amount of mucus becomes thick and difficult to clear > leads to recurring infections > scarring
High sodium sweat - don’t have a high blood-salt level as it’s lost in sweat > higher risk of dehydration
Pancreatic insufficiency - abnormal ion transport means enzyme secretions are dehydrated
Biliary disease - bile duct too concentrated and damages lumen
Infertility (males) - Absence of vas deferens
Cirrhosis of the liver - high concentrated secretions lead to liver damage
Gastrointestinal disease - Low volume of secretions, changed of fluid movement, dehydration > problems with bowel movements

Question 4

Symptoms of CF (lungs)

Answer 4

• Persistent cough
• Coughing fits
• Inflammation in the lungs - wheezing, SOB, Dyspnoea
• Recurring chest and lung infections
• CF cross infection can occur
• Large, smell stools
• Malnutrition

Question 5

CF physio treatment

Answer 5

Postural drainage, percussion, shakes/vibs, ACBT, autogenic drainage, adjuncts, mobilisiation/exercise, suction