Cystic Fibrosis

Question 1

What is CF?

Answer 1

• Purely genetic
• Affects the lungs and the pancreas
• Can cause other complications and affect other organs

Question 2

What is the class I mutation for CF?

Answer 2

No protein is made

Question 3

What is the class II mutation for CF?

Answer 3

Protein is abnormal and destroyed before it reaches membrane

Question 4

What is the class III mutation for CF?

Answer 4

Protein reaches membrane but channel is blocked

Question 5

What is the class IV mutation for CF?

Answer 5

Protein reaches membrane but channel does not work effectively

Question 6

What is the class V mutation for CF?

Answer 6

Protein reaches membrane and works properly but not many are made

Question 7

Why is thick mucus a problem?

Answer 7

Thick mucus can block several organs:
•Pancreas
•Sinuses 
•Reproductive 
•Intestines
•Airways in the lungs
Thick mucus can accumulate, get infected and cause inflammation. The perfect storm for bacterial growth!

Question 8

What are the symptoms of CF?

Answer 8

§ Persistent cough
§ Difficulty with growth § Abdominal pain
§ Bloated stomach
§ Salty tasting skin
§ Clubbing of fingers

Question 9

What are the oral pancreatic enzymes used by CF patients? How are they adjusted?

Answer 9

• Different brands available: Creon 5,10, 25, Pancrease,
  Viokase, Ultrase, etc..
• They contain lipase, protease and amylase
• Need to be taken just before eating
• Number of pills may need to be adjusted depending on the meal/snack

Question 10

What are other medications used by CF patients?

Answer 10

• Pulmozyme
• Tobramycin (TOBI) and Cayston
• Azithromycin
• Bronchodilators or corticosteroids
• Multivitamins
• Vitamin D and Calcium
• Iron supplements, Zinc, Omega-3 as required
• Prednisone as needed
• Lax-a-day, nasal wash as needed

Question 11

What are the complications in CF?

Answer 11

• Frequent infections requiring hospitalization
• Nutrient deficiencies due to malabsorption
• Gastro-oesophageal reflux disease
• Inflammatory Bowel diseases
• Osteoporosis
• Intestinal obstruction (DIOS)
• Liver disease (ex: fatty liver) n Kidney disease
• Heart problems
• CF related diabetes

Question 12

What is Distal Intestinal Obstructive Syndrome (DIOS)?

Answer 12

• Risk factors include severe genotype, pancreatic insufficiency, fat malabsorption, dehydration, history of meconium ileus or DIOS, organ transplantation, etc.
• Symptoms include acute onset of severe abdominal pain, nausea, vomiting (including bilious material) and inability to pass gas or stool.

Question 13

What are the treatment options for incomplete DIOS?

Answer 13

Oral rehydration with stool softeners and laxatives (ex. PegLyte)

Question 14

What are the treatment options for complete DIOS?

Answer 14

• IV rehydration
• Nasogastric aspiration
• Enemas, laxatives
• Surgery may be required

Question 15

What are the considerations after DIOS?

Answer 15

• Patient may have anxiety towards certain foods or eating in general
• Changes may need to be made on enzyme dosage
• Patient may need to keep track of their water or fiber intake
• Patients may have to add laxatives to their usual routine

Question 16

What are the energy requirements for CF?

Answer 16

• Energy requirement is said to be 110%-200% of that of a healthy individual of same age and gender
• Energy requirements are higher due to many factors like inflammation, malabsorption, the presence of complications, etc.
• Research suggests a positive association between BMI and lung function.

Question 17

What are the factors that influence eating in CF?

Answer 17

• Presence of Dyspnea
• Sinus problems
• Medications (steroids)
• Presence of GI problems u Slow intestinal motility
• History of DIOS

Question 18

What are the nutrition recommendations for CF patients?

Answer 18

• High protein, High Energy Diet
• Teach patients/parents ways to increase energy/protein in each recipes (ex: adding milk powder to shakes, adding cream in soups)
• Supplements added to diet as snacks (Scandishake, Ensure plus, Boost, etc.)
• Enteral nutrition may be necessary

Question 19

What are the new cures available for CF?

Answer 19

Kalydeco (ivacaftor) has been approved for 11 different mutations, 10 more than 2012 when it was first approved by Health Canada.

Orkambi (ivacaftor/lumacaftor) is a new drug combination to help patients with 2 copies of the common F508del mutation. It has been approved by Health Canada in 2016.