Cystic Fibrosis Overview and Symptoms
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Questions and Answers

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?

  • 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?

  • 40s
  • 30s
  • 50s (correct)
  • 80s

Which treatment is used to prevent lung infections in individuals with Cystic Fibrosis?

<p>Antibiotics (B)</p> Signup and view all the answers

What effect does Cystic Fibrosis have on the mucus produced in the body?

<p>It becomes thick and obstructive (B)</p> Signup and view all the answers

What is a common complication associated with Cystic Fibrosis in males?

<p>Male infertility (D)</p> Signup and view all the answers

Which treatment option is a CFTR modulator?

<p>CFTR modulators (C)</p> Signup and view all the answers

What is a common measure for breathing support in individuals with Cystic Fibrosis?

<p>Oxygen mask (C)</p> Signup and view all the answers

Which modulator is primarily used for people with Cystic Fibrosis aged 2 and older?

<p>Elexacaftor-Tezacaftor-Ivacaftor (A)</p> 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?

<p>25% (A)</p> Signup and view all the answers

At what age is the combination of Tezacaftor and Ivacaftor approved for use?

<p>4 months (C)</p> Signup and view all the answers

Which transplantation option is mentioned for individuals with potentially severe lung disease?

<p>Lung transplant (C)</p> Signup and view all the answers

What percentage of people are diagnosed with Cystic Fibrosis by the age of 2?

<p>75% (B)</p> Signup and view all the answers

Which population is noted to be less likely to develop Cystic Fibrosis?

<p>African (C), Asian (D)</p> Signup and view all the answers

What is one possible disability associated with Cystic Fibrosis?

<p>Diabetes (D)</p> Signup and view all the answers

Which age group is Ivacaftor approved for in treating Cystic Fibrosis?

<p>Adults and children 4 months and older (A)</p> Signup and view all the answers

Cystic Fibrosis is a disorder that primarily affects the lungs and digestive system.

<p>True (A)</p> Signup and view all the answers

Cystic Fibrosis is caused by a gene called the Cystic Fibrosis Receptor Transmembrane Modulator.

<p>False (B)</p> Signup and view all the answers

Individuals with Cystic Fibrosis may experience symptoms such as poor growth and lung infections.

<p>True (A)</p> Signup and view all the answers

The average lifespan of individuals with Cystic Fibrosis is in the 30s.

<p>False (B)</p> Signup and view all the answers

People with Cystic Fibrosis only inherit the disease if they have two copies of the defective CFTR gene.

<p>True (A)</p> Signup and view all the answers

Bronchodilators are used in the treatment of Cystic Fibrosis to shrink the airways.

<p>False (B)</p> Signup and view all the answers

Elevated risk of diabetes is a common complication associated with Cystic Fibrosis.

<p>True (A)</p> Signup and view all the answers

Cystic Fibrosis primarily affects older adults and does not appear in childhood.

<p>False (B)</p> Signup and view all the answers

Elexacaftor-Tezacaftor-Ivacaftor is approved for children aged 3 and older.

<p>False (B)</p> Signup and view all the answers

Liver transplants can be an option for advanced diseases like cirrhosis.

<p>True (A)</p> Signup and view all the answers

The probability of a child having Cystic Fibrosis if both parents are carriers is 50%.

<p>False (B)</p> Signup and view all the answers

People with CF can still manage to have a job and go to school.

<p>True (A)</p> Signup and view all the answers

Combining Tezacaftor and Ivacaftor is not approved for children under 6 months.

<p>True (A)</p> Signup and view all the answers

A lung transplant can help individuals with severe lung disease.

<p>True (A)</p> Signup and view all the answers

75% of people with CF are diagnosed after the age of 10.

<p>False (B)</p> Signup and view all the answers

Combination of Lumacaftor and Ivacaftor is approved for adults only.

<p>False (B)</p> Signup and view all the answers

Flashcards

Cystic Fibrosis

An inherited disease causing thick mucus build-up, primarily affecting lungs and digestive system.

CFTR Gene

The gene responsible for cystic fibrosis. Mutations in this gene lead to the disease.

What causes Cystic Fibrosis?

Inherited mutations in the CFTR gene, specifically two copies of the mutated gene.

CF Symptoms

Thick mucus buildup leading to lung infections, digestive issues, and salty skin.

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CF Prognosis

Most people with CF can live a mostly normal life with proper treatment. Average lifespan is in the 50s, but some can live to their 80s.

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Antibiotics for CF

Used to prevent and treat lung infections, a common complication in CF.

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Bronchodilators for CF

Medications that relax and open airways, improving breathing.

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CFTR Modulators

These medications help the faulty CFTR protein work better, improving the disease's effects.

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Elexacaftor-Tezacaftor-Ivacaftor

A combination therapy approved for people with CF aged 2 and older, effective in up to 90% of cases.

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Lumacaftor and Ivacaftor

A combination therapy approved for people with CF aged 1 and older.

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Tezacaftor and Ivacaftor

A combination therapy approved for people with CF aged 4 months and older.

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Lung Transplant for CF

A surgical option considered for individuals with severe lung disease and respiratory failure caused by CF.

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CF Gene Carrier Probability

If both parents carry the CF gene, there's a 25% chance their child will have CF.

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CF Prevalence in Canada

Approximately 1 in 3000 Canadians have CF, with a higher chance in certain racial groups.

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Life with CF

Individuals with CF can still live full lives, including having children, working, and going to school.

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Lung Transplant

A surgical option considered for CF patients with severe lung disease and respiratory failure.

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What is Cystic Fibrosis?

Cystic fibrosis (CF) is an inherited disorder that affects the cells producing mucus, sweat, and digestive juices. A defective gene causes these fluids to become thick, leading to blockages in ducts and passageways, primarily in the lungs and pancreas.

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How is CF Inherited?

CF is an inherited disease caused by mutations in the CFTR gene. A person must inherit two copies of the mutated gene, one from each parent, to develop CF.

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Common CF Symptoms

Common symptoms of CF include persistent coughing, lung infections, difficulty breathing, wheezing, salty skin, poor weight gain, and digestive problems.

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Lung Infections in CF

People with CF are prone to frequent lung infections because the thick mucus traps bacteria, leading to pneumonia and bronchitis.

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CF Treatments

Treatments for CF aim to manage symptoms, prevent complications, and improve lung function. They include antibiotics, bronchodilators, inhaled medications, and CFTR modulators.

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What are CFTR Modulators?

CFTR modulators are medications that help the faulty CFTR protein function better, reducing the negative effects of the gene mutation.

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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.

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