Cystic Fibrosis

Question 1

What is cystic fibrosis?

Answer 1

Genetic disorder that affects the respiratory, digestive and reproductive symptoms

Question 2

Characteristics of Cystic Fibrosis

Answer 2

-Excessively viscis mucus leading to obstruction of pathways
-increased Na+ and CL- content in sweat

Question 3

How did they first test for cystic fibrosis in babies

Answer 3

They licked them

Question 4

What race is Cystic Fibrosis the most common life-threatening disease?

Answer 4

Caucasian

Question 5

How is CF diagnosed among Gender?

Answer 5

-Equally diagnosed in m/f
-Males have longer life expectancy

Question 6

How much of CF patients are younger than 18 years old?

Answer 6

53%

Question 7

What gene is responsible for genetic inheritance of CF

Answer 7

CFTR gene (Chromosome 7)

Gene is Autosomal Recessive

Question 8

What does a genetic mutation on chromosome 7 do to the pathophysiology of CF?

Answer 8

-creates abnormal CFTR channel (abnormal NACl and H20 movement across cell membrane)

-mucus becomes abnormally thick and dry

Question 9

What is the pathophysiological cascade in a respiratory system with CF present?

Answer 9

1. Bronchial airway obstruction
2. Bacterial infections (staphylococcus aureus)
3. Inflammation

Question 10

What are some CF implications on the GI tract?

Answer 10

1. Exocrine pancreatic insufficiency (malabsorption of nutrients)
2. Endocrine pancreatic insufficiency (diabetes)
3. Liver & Gallbladder Disease

Question 11

In the GI Tract with a patient with CF, what are some symptoms associated with Liver & Gallbladder Disease?

Answer 11

Channel Defects, Obstruction of bile ducts, Increased # of Gallstones

Question 12

What happens to the heart with CF?

Answer 12

Becomes enlarged

Question 13

What are some clinical symptoms of CF?

(Respiratory, GI, Metabolic, etc.)

Answer 13

Question 14

Visual signs an symptoms of CF?

Answer 14

Digital clubbing

Question 15

What is the most important consideration before exercise testing a patient with CF?

Answer 15

Level of pulmonary disease

Question 16

What are right heart failure symptoms?

Answer 16

Edema
Hypoxemia

Question 17

What is the most prominent symptom of liver disease in CF patients?

Answer 17

Ascites (abdominal swelling)

Question 18

What are some symptoms of CF-related diabetes?

Answer 18

(Hyper / Hypo) - glycemia
Excessive fatigue
Confusion
Dizziness

Question 19

CF patients are at _______ risk of dehydration?

Answer 19

increased

Question 20

What are orthopedic factors associated with CF?

Answer 20

-Postural deformities
-Increased thoracic kyphosis
-hamstring inflexibility

Question 21

Decreased bone mineralization in CF patients increases the risk for ________

Answer 21

Osteoporosis

Question 22

What is the diagnostic test used for CF?

Answer 22

Sweat chloride test or genetic mutation analysis

Question 23

What are the cutoffs for the Sweat Chloride test in infants?

Answer 23

< 29 mmol/L (negative) = CF is very unlikely

30 - 59 mmol/L (intermediate) = CF is possible

> 60 mmol/L (positive) = CF is likely to be diagnosed

Question 24

What are the cutoffs for the Sweat Chloride test in people older than 6 months?

Answer 24

< 39 mmol/L (negative) = CF is very unlikely

40 - 59 mmol/L (intermediate) = CF is possible

> 60 mmol/L (positive) = CF is likely to be diagnosed

Question 25

What are other diagnosic tests used for CF? (excluding sweat chloride test)

Answer 25

Sputum culture
Chest radiograph
CT scan

Question 26

What is the most important thing about PFT?

Answer 26

Assesses changes over time

Question 27

Exercise GXT testing accommodations for CF patients? (Supplemental O2)

Answer 27

> 90% - supplemental
oxygen is unnecessary
<90% - supplemental
oxygen may be necessary

Question 28

What is improved with treatment of CF

Answer 28

Average life expectancy

Question 29

Precautions for Postural Drainage

Answer 29

Question 30

Precautions for Percussion and Vibration?

Answer 30

Question 31

What do airway devices do for CF patients?

Answer 31

Flutter valve that allows the patient to loosen mucus

Question 32

Why are chest wall oscillation devices used for treatment of CF?

Answer 32

-For clearing excess mucus
-oscillations thin out airway mucus

Question 33

Exercise goals with CF patients

Answer 33

-Enhance physical fitness
-Reduce severity of symptoms
-Safe and enjoyable participation

Question 34

What are special considerations with exercise for CF patients?

Answer 34

CF-related diabetes
Exercise-induced bronchospasm

Question 35

Aerobic FIIT guidelines for CF

Answer 35

F: 3-5 days/wk (children/adolescents) | 5 days (adults)

I: 70%-85% of HR max (Lower for beginners)

T: 20-60 min

T: NO specific modality is optimal

Question 36

Resistance FIIT guidelines for CF

Answer 36

F: 3-5 days/week

I: 50-60% 1RM 3x12
(children = high intensity) (adults = physician consultation)

T: (children = 10-30 min) (adults = 30 min
combined strength and inspiratory muscle training)

T: free weights, machines, body weight

Question 37

Flexibility training FIIT guidelines

Answer 37

2 days/wk
Before & After activity

Question 38

Main exercise targets for CF

Answer 38

Endurance
Chest mobility
Core strengthening
Leg strengthening
Breathing exercises

Question 39

Exercise for babies and toddlers

Answer 39

Question 40

Exercises for Preschoolers and School-aged children

Answer 40

Question 41

Exercise for teenagers and adults

Answer 41

Question 42

CF patients are in a chronic energy ______

Answer 42

Deficit

(110-200% energy needs for same age, gender, size)