Podcast
Questions and Answers
What primarily causes Cystic Fibrosis?
What primarily causes Cystic Fibrosis?
Which of the following is NOT a common symptom of Cystic Fibrosis?
Which of the following is NOT a common symptom of Cystic Fibrosis?
What is the average lifespan of an individual with Cystic Fibrosis?
What is the average lifespan of an individual with Cystic Fibrosis?
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?
Signup and view all the answers
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?
Signup and view all the answers
What is a common complication associated with Cystic Fibrosis in males?
What is a common complication associated with Cystic Fibrosis in males?
Signup and view all the answers
Which treatment option is a CFTR modulator?
Which treatment option is a CFTR modulator?
Signup and view all the answers
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?
Signup and view all the answers
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?
Signup and view all the answers
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?
Signup and view all the answers
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?
Signup and view all the answers
Which transplantation option is mentioned for individuals with potentially severe lung disease?
Which transplantation option is mentioned for individuals with potentially severe lung disease?
Signup and view all the answers
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?
Signup and view all the answers
Which population is noted to be less likely to develop Cystic Fibrosis?
Which population is noted to be less likely to develop Cystic Fibrosis?
Signup and view all the answers
What is one possible disability associated with Cystic Fibrosis?
What is one possible disability associated with Cystic Fibrosis?
Signup and view all the answers
Which age group is Ivacaftor approved for in treating Cystic Fibrosis?
Which age group is Ivacaftor approved for in treating Cystic Fibrosis?
Signup and view all the answers
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.
Signup and view all the answers
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.
Signup and view all the answers
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.
Signup and view all the answers
The average lifespan of individuals with Cystic Fibrosis is in the 30s.
The average lifespan of individuals with Cystic Fibrosis is in the 30s.
Signup and view all the answers
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.
Signup and view all the answers
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.
Signup and view all the answers
Elevated risk of diabetes is a common complication associated with Cystic Fibrosis.
Elevated risk of diabetes is a common complication associated with Cystic Fibrosis.
Signup and view all the answers
Cystic Fibrosis primarily affects older adults and does not appear in childhood.
Cystic Fibrosis primarily affects older adults and does not appear in childhood.
Signup and view all the answers
Elexacaftor-Tezacaftor-Ivacaftor is approved for children aged 3 and older.
Elexacaftor-Tezacaftor-Ivacaftor is approved for children aged 3 and older.
Signup and view all the answers
Liver transplants can be an option for advanced diseases like cirrhosis.
Liver transplants can be an option for advanced diseases like cirrhosis.
Signup and view all the answers
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%.
Signup and view all the answers
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.
Signup and view all the answers
Combining Tezacaftor and Ivacaftor is not approved for children under 6 months.
Combining Tezacaftor and Ivacaftor is not approved for children under 6 months.
Signup and view all the answers
A lung transplant can help individuals with severe lung disease.
A lung transplant can help individuals with severe lung disease.
Signup and view all the answers
75% of people with CF are diagnosed after the age of 10.
75% of people with CF are diagnosed after the age of 10.
Signup and view all the answers
Combination of Lumacaftor and Ivacaftor is approved for adults only.
Combination of Lumacaftor and Ivacaftor is approved for adults only.
Signup and view all the answers
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
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Related Documents
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.
