Podcast
Questions and Answers
What primarily causes Cystic Fibrosis?
What primarily causes Cystic Fibrosis?
- Exposure to environmental toxins
- Dietary deficiencies
- Mutations in the CFTR gene (correct)
- Viral infections during childhood
Which of the following is NOT a common symptom of Cystic Fibrosis?
Which of the following is NOT a common symptom of Cystic Fibrosis?
- Wheezing or shortness of breath
- Chronic sinus infections
- Frequent headaches (correct)
- Excessive sweating leading to dehydration
What is the average lifespan of an individual with Cystic Fibrosis?
What is the average lifespan of an individual with Cystic Fibrosis?
- 40s
- 30s
- 50s (correct)
- 80s
Which treatment is used to prevent lung infections in individuals with Cystic Fibrosis?
Which treatment is used to prevent lung infections in individuals with Cystic Fibrosis?
What effect does Cystic Fibrosis have on the mucus produced in the body?
What effect does Cystic Fibrosis have on the mucus produced in the body?
What is a common complication associated with Cystic Fibrosis in males?
What is a common complication associated with Cystic Fibrosis in males?
Which treatment option is a CFTR modulator?
Which treatment option is a CFTR modulator?
What is a common measure for breathing support in individuals with Cystic Fibrosis?
What is a common measure for breathing support in individuals with Cystic Fibrosis?
Which modulator is primarily used for people with Cystic Fibrosis aged 2 and older?
Which modulator is primarily used for people with Cystic Fibrosis aged 2 and older?
What is the chance of a child having Cystic Fibrosis if both parents are carriers of the CF gene?
What is the chance of a child having Cystic Fibrosis if both parents are carriers of the CF gene?
At what age is the combination of Tezacaftor and Ivacaftor approved for use?
At what age is the combination of Tezacaftor and Ivacaftor approved for use?
Which transplantation option is mentioned for individuals with potentially severe lung disease?
Which transplantation option is mentioned for individuals with potentially severe lung disease?
What percentage of people are diagnosed with Cystic Fibrosis by the age of 2?
What percentage of people are diagnosed with Cystic Fibrosis by the age of 2?
Which population is noted to be less likely to develop Cystic Fibrosis?
Which population is noted to be less likely to develop Cystic Fibrosis?
What is one possible disability associated with Cystic Fibrosis?
What is one possible disability associated with Cystic Fibrosis?
Which age group is Ivacaftor approved for in treating Cystic Fibrosis?
Which age group is Ivacaftor approved for in treating Cystic Fibrosis?
Cystic Fibrosis is a disorder that primarily affects the lungs and digestive system.
Cystic Fibrosis is a disorder that primarily affects the lungs and digestive system.
Cystic Fibrosis is caused by a gene called the Cystic Fibrosis Receptor Transmembrane Modulator.
Cystic Fibrosis is caused by a gene called the Cystic Fibrosis Receptor Transmembrane Modulator.
Individuals with Cystic Fibrosis may experience symptoms such as poor growth and lung infections.
Individuals with Cystic Fibrosis may experience symptoms such as poor growth and lung infections.
The average lifespan of individuals with Cystic Fibrosis is in the 30s.
The average lifespan of individuals with Cystic Fibrosis is in the 30s.
People with Cystic Fibrosis only inherit the disease if they have two copies of the defective CFTR gene.
People with Cystic Fibrosis only inherit the disease if they have two copies of the defective CFTR gene.
Bronchodilators are used in the treatment of Cystic Fibrosis to shrink the airways.
Bronchodilators are used in the treatment of Cystic Fibrosis to shrink the airways.
Elevated risk of diabetes is a common complication associated with Cystic Fibrosis.
Elevated risk of diabetes is a common complication associated with Cystic Fibrosis.
Cystic Fibrosis primarily affects older adults and does not appear in childhood.
Cystic Fibrosis primarily affects older adults and does not appear in childhood.
Elexacaftor-Tezacaftor-Ivacaftor is approved for children aged 3 and older.
Elexacaftor-Tezacaftor-Ivacaftor is approved for children aged 3 and older.
Liver transplants can be an option for advanced diseases like cirrhosis.
Liver transplants can be an option for advanced diseases like cirrhosis.
The probability of a child having Cystic Fibrosis if both parents are carriers is 50%.
The probability of a child having Cystic Fibrosis if both parents are carriers is 50%.
People with CF can still manage to have a job and go to school.
People with CF can still manage to have a job and go to school.
Combining Tezacaftor and Ivacaftor is not approved for children under 6 months.
Combining Tezacaftor and Ivacaftor is not approved for children under 6 months.
A lung transplant can help individuals with severe lung disease.
A lung transplant can help individuals with severe lung disease.
75% of people with CF are diagnosed after the age of 10.
75% of people with CF are diagnosed after the age of 10.
Combination of Lumacaftor and Ivacaftor is approved for adults only.
Combination of Lumacaftor and Ivacaftor is approved for adults only.
Flashcards
Cystic Fibrosis
Cystic Fibrosis
An inherited disease causing thick mucus build-up, primarily affecting lungs and digestive system.
CFTR Gene
CFTR Gene
The gene responsible for cystic fibrosis. Mutations in this gene lead to the disease.
What causes Cystic Fibrosis?
What causes Cystic Fibrosis?
Inherited mutations in the CFTR gene, specifically two copies of the mutated gene.
CF Symptoms
CF Symptoms
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CF Prognosis
CF Prognosis
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Antibiotics for CF
Antibiotics for CF
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Bronchodilators for CF
Bronchodilators for CF
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CFTR Modulators
CFTR Modulators
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Elexacaftor-Tezacaftor-Ivacaftor
Elexacaftor-Tezacaftor-Ivacaftor
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Lumacaftor and Ivacaftor
Lumacaftor and Ivacaftor
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Tezacaftor and Ivacaftor
Tezacaftor and Ivacaftor
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Lung Transplant for CF
Lung Transplant for CF
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CF Gene Carrier Probability
CF Gene Carrier Probability
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CF Prevalence in Canada
CF Prevalence in Canada
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Life with CF
Life with CF
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Lung Transplant
Lung Transplant
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What is Cystic Fibrosis?
What is Cystic Fibrosis?
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How is CF Inherited?
How is CF Inherited?
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Common CF Symptoms
Common CF Symptoms
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Lung Infections in CF
Lung Infections in CF
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CF Treatments
CF Treatments
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What are CFTR Modulators?
What are CFTR Modulators?
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Study Notes
Cystic Fibrosis Overview
- Cystic fibrosis (CF) is an inherited disorder
- It causes severe damage to the lungs, digestive system, and other organs
- CF affects cells that produce mucus, sweat, and digestive juices
- Defective CF gene causes these fluids to become thick
- Thick fluids block ducts and passageways, especially in the lungs and pancreas
- CF can lead to a range of complications, such as difficulty breathing, digestive problems, and infections
Causes of Cystic Fibrosis
- CF is inherited through mutations in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR)
- People who inherit two copies of the mutated CFTR gene are at risk for CF
- Having the gene doesn't guarantee symptoms; some people carry the gene but aren't affected.
- CF is commonly associated with mutations in the CFTR gene.
Symptoms of Cystic Fibrosis
- Respiratory: Lung infections (pneumonia, bronchitis), coughing, wheezing or shortness of breath, nasal polyps, chronic sinus infections
- Digestive: Difficulty with bowel movements, poor growth or weight gain, salty-tasting skin, clubbing of fingers and toes
- Other: Male infertility, elevated risk of diabetes
Prognosis of Cystic Fibrosis
- Most people with CF can live mostly normal lives, attending school and post-secondary education
- Average lifespan is in the 50s, but some live into their 80s
- Advances in treatments and care have dramatically improved outcomes for people with CF.
Treatments for Cystic Fibrosis
- Medicine: Antibiotics to prevent lung infections, anti-inflammatory medicine (like ibuprofen or corticosteroids), bronchodilators to open airways
- Breathing Support: Oxygen mask, ventilator support to moisten air and deliver it to the lungs
- CFTR Modulators: Drugs that improve how faulty CFTR protein works. Different modulators help different types of mutations. Some examples include Elexacaftor-Tezacaftor-Ivacaftor, Ivacaftor, combinations of Lumacaftor or Tezacaftor with Ivacaftor.
- Surgery: Lung transplant or liver transplant may be options for severe cases with advanced disease.
Facts about Cystic Fibrosis
- CF affects 105,000 children and adults globally
- Approximately 10,000,000 people are symptom-free carriers
- The chance of having CF if both parents are carriers is 25%. A 50% chance of being a carrier, and 25% of not being a carrier.
- The risk of CF is similar for males and females
- Estimated 1 in 3000 Canadians have CF
Support Groups in Canada
- Cystic Fibrosis Canada (CF.ca, 1-800-378-2233)
- Lung Saskatchewan support groups
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Description
This quiz covers the essential aspects of cystic fibrosis, an inherited disorder that severely affects the lungs and digestive system. Learn about its causes, symptoms, and the impact of the defective CF gene on bodily functions. Test your knowledge on this complex condition.