20. CF patient experience

Question 1

what is cystic fibrosis

Answer 1

an inherited, chronic disease that affects primarily the lungs and digestive tract

Question 2

what causes CF

Answer 2

a mutation in the CFTR gene

Question 3

what is the effect of CF

Answer 3

the thickening and build up of mucous

Question 4

explain CF diagnosis

Answer 4

new born screening: involves collecting a small blood sample from the newborn’s heel and testing it for elevated levels of immunoreactive trypsinogen (IRT), a protein that may indicate CF.
A positive newborn screening result does not confirm CF but indicates a need for further testing

The sweat test is the gold standard diagnostic test for CF. Elevated levels of chloride in the sweat confirm the diagnosis of CF.

Question 5

when was CF added to the newborn screening panel

Answer 5

2011

Question 6

what is the difference in outcome for severe and mild CF

Answer 6

mild CF has 56 years predicted survival

severe CF has 37 years predicted survival

Question 7

what are the symptoms of CF

Answer 7

• persistent coughing
• shortness of breath
• chronic lung infection
• poor growth
• salty- tasting skin
• greasy/bulky stools

Question 8

Lung therapies due to CF

Answer 8

• inhalers: to open airways
• oral antibiotics: to prevent chest infections
• nebulizers
• physio: to help clear mucus
• sinus rinses: to flush mucus
• supplemental oxygen: used when blood saturation is low

Question 9

what are nebulisers

Answer 9

devices that change liquid medication into a mist to be inhaled through a mask or mouthpiece

Question 10

Gastrointestinal therapies for CF

Answer 10

• high calorie diet: to obtain enough nutrients and vitamins
• taking enzymes: to assist in digesting food
• PEG feed: method of supplemental feeding to help gain weight
• taking multivitamins and supplements
• taking osmotic laxatives: prevent constipation
• anti reflux medication:

Question 11

cystic fibrosis related diabetes (CFRD)

Answer 11

unique form of diabetes that affects approximately 40% of people with CF

patients with CFRD must monitor their glucose levels on a regular basis

Question 12

day in the life of a person with CF

Answer 12

• morning begins with coughing out mucus that has accumulated overnight
• nebulizers to open airways
• chest clearing and physio
• takes prescribed medication
• high calorie lunch
• daily exercise
• high calorie dinner
• nubulised treatments, chest clearance physio