Cystic Fibrosis (signs/symptoms, treatment, FITT) Flashcards
What is cystic fibrosis?
Genetic disorder that affects the respiratory, digestive and reproductive symptoms
Characteristics of Cystic Fibrosis
-Excessively viscis mucus leading to obstruction of pathways
-increased Na+ and CL- content in sweat
How did they first test for cystic fibrosis in babies
They licked them
What race is Cystic Fibrosis the most common life-threatening disease?
Caucasian
How is CF diagnosed among Gender?
-Equally diagnosed in m/f
-Males have longer life expectancy
How much of CF patients are younger than 18 years old?
53%
What gene is responsible for genetic inheritance of CF
CFTR gene (Chromosome 7)
Gene is Autosomal Recessive
What does a genetic mutation on chromosome 7 do to the pathophysiology of CF?
-creates abnormal CFTR channel (abnormal NACl and H20 movement across cell membrane)
-mucus becomes abnormally thick and dry
What is the pathophysiological cascade in a respiratory system with CF present?
- Bronchial airway obstruction
- Bacterial infections (staphylococcus aureus)
- Inflammation
What are some CF implications on the GI tract?
- Exocrine pancreatic insufficiency (malabsorption of nutrients)
- Endocrine pancreatic insufficiency (diabetes)
- Liver & Gallbladder Disease
In the GI Tract with a patient with CF, what are some symptoms associated with Liver & Gallbladder Disease?
Channel Defects, Obstruction of bile ducts, Increased # of Gallstones
What happens to the heart with CF?
Becomes enlarged
What are some clinical symptoms of CF?
(Respiratory, GI, Metabolic, etc.)
Visual signs an symptoms of CF?
Digital clubbing
What is the most important consideration before exercise testing a patient with CF?
Level of pulmonary disease
What are right heart failure symptoms?
Edema
Hypoxemia
What is the most prominent symptom of liver disease in CF patients?
Ascites (abdominal swelling)
What are some symptoms of CF-related diabetes?
(Hyper / Hypo) - glycemia
Excessive fatigue
Confusion
Dizziness
CF patients are at _______ risk of dehydration?
increased
What are orthopedic factors associated with CF?
-Postural deformities
-Increased thoracic kyphosis
-hamstring inflexibility
Decreased bone mineralization in CF patients increases the risk for ________
Osteoporosis
What is the diagnostic test used for CF?
Sweat chloride test or genetic mutation analysis
What are the cutoffs for the Sweat Chloride test in infants?
< 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
What are the cutoffs for the Sweat Chloride test in people older than 6 months?
< 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
What are other diagnosic tests used for CF? (excluding sweat chloride test)
Sputum culture
Chest radiograph
CT scan
What is the most important thing about PFT?
Assesses changes over time
Exercise GXT testing accommodations for CF patients? (Supplemental O2)
> 90% - supplemental
oxygen is unnecessary
<90% - supplemental
oxygen may be necessary
What is improved with treatment of CF
Average life expectancy
Precautions for Postural Drainage
Precautions for Percussion and Vibration?
What do airway devices do for CF patients?
Flutter valve that allows the patient to loosen mucus
Why are chest wall oscillation devices used for treatment of CF?
-For clearing excess mucus
-oscillations thin out airway mucus
Exercise goals with CF patients
-Enhance physical fitness
-Reduce severity of symptoms
-Safe and enjoyable participation
What are special considerations with exercise for CF patients?
CF-related diabetes
Exercise-induced bronchospasm
Aerobic FIIT guidelines for CF
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
Resistance FIIT guidelines for CF
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
Flexibility training FIIT guidelines
2 days/wk
Before & After activity
Main exercise targets for CF
Endurance
Chest mobility
Core strengthening
Leg strengthening
Breathing exercises
Exercise for babies and toddlers
Exercises for Preschoolers and School-aged children
Exercise for teenagers and adults
CF patients are in a chronic energy ______
Deficit
(110-200% energy needs for same age, gender, size)
