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Introduction

- Cystic Fibrosis (CF) is a genetic disorder that affects the exocrine glands, which produce mucus, sweat, and digestive enzymes.

- CF causes these secretions to become thick and sticky, leading to blockage of the airways, lungs, pancreas, liver, and other organs.

- CF can cause chronic respiratory infections, malnutrition, diabetes, infertility, and other complications.

- CF is inherited in an autosomal recessive pattern, meaning that both parents must carry a mutated copy of the CFTR gene for their child to have the disease.

- There is no cure for CF, but treatments can help manage the symptoms and improve the quality of life of people with CF.

Questions on Introduction

Correct Answer is B

Explanation

Incorrect. CF is primarily a genetic disorder with well-established genetic mutations associated with its development.

Correct Answer is B

Explanation

Incorrect. High blood pressure is not a characteristic symptom of CF.

Correct Answer is B

Explanation

Incorrect. CF affects both the bronchial tubes and the entire respiratory system, often leading to chronic lung infections.

Correct Answer is C

Explanation

Incorrect. CF primarily affects the pancreas and small intestine rather than the large intestine, and chronic constipation is not a typical symptom of CF.

Correct Answer is C

Explanation

Incorrect. The risk of a child having CF is determined by both parents' genetic history, not solely the mother's.

Correct Answer is C

Explanation

Incorrect. CF is primarily a genetic disorder with well-established genetic mutations associated with its development.

Correct Answer is D

Explanation

Correct. The CFTR protein is responsible for regulating mucus consistency and chloride ion transport in the respiratory and digestive systems.

Correct Answer is C

Explanation

Incorrect. CF is not characterized by the absence of the CFTR gene; instead, it involves various mutations in this gene.

Correct Answer is C

Explanation

Incorrect. The production of digestive enzymes in the pancreas is not a direct function of the CFTR protein.

Correct Answer is B

Explanation

Incorrect. The CFTR protein does not produce oxygen but rather regulates chloride ion and water transport in the lungs

Correct Answer is A

Explanation

Incorrect. CF is not caused by exposure to respiratory pathogens during infancy; it is primarily a genetic disorder.

Correct Answer is C

Explanation

Incorrect. While CF can affect individuals from any ethnicity, it is primarily a genetic disorder, and family history plays a crucial role in the risk of inheritance.

Correct Answer is D

Explanation

Correct. When both parents are carriers, there is a 25% chance that their child will inherit two CF genes and have CF, a 50% chance of being a carrier, and a 25% chance of inheriting two normal genes (no CF).

Correct Answer is B

Explanation

Incorrect. Genetic testing is not reserved solely for family members of individuals already diagnosed with CF; it is used for diagnostic purposes in suspected cases of CF.

Correct Answer is B

Explanation

Incorrect. Digestive discomfort and diarrhea are more related to CF's impact on the gastrointestinal system, rather than being early respiratory symptoms.

Correct Answer is B

Explanation

Incorrect. Muscle weakness is not a common gastrointestinal symptom in CF but may be associated with other complications.

Correct Answer is B

Explanation

Incorrect. While CF can affect the digestive system, clubbing of the fingers is primarily associated with respiratory issues.

Incorrect. Rapid weight gain is not associated with advanced CF-related lung disease; individuals with advanced CF often experience weight loss and malnutrition.

Incorrect. Rapid growth and development are typically observed in adolescents, not as a complication but as a part of normal growth.

Incorrect. A complete blood count (CBC) is a general blood test and is not specific for CF diagnosis.

Incorrect. Pulmonary function tests are used to evaluate lung function but are not typically the initial diagnostic test for CF in infants.

Correct. Pulmonary function tests are commonly performed to assess lung function, monitor disease progression, and guide treatment in individuals with CF.

Correct. Genetic testing is a diagnostic procedure commonly used to confirm CF by analyzing the specific CF gene mutations present in an individual.

Correct. The sweat chloride test measures the concentration of chloride ions in sweat and is commonly used to confirm CF. Elevated sweat chloride levels are indicative of CF.

Correct. High-frequency chest wall oscillation (HFCWO) involves using a mechanical device to deliver oscillations to the chest wall, helping to mobilize and clear mucus from the airways.

Incorrect. Restricting fluid intake is not recommended for individuals with CF, as staying hydrated is important for preventing thickening of mucus.

Incorrect. Bed rest and minimal physical activity are not recommended as the primary treatment for pulmonary exacerbations; maintaining mobility and airway clearance techniques are important.

Correct. The primary goal of airway clearance techniques in CF is to mobilize and clear mucus and secretions from the airways, improving breathing and reducing the risk of infection.

Incorrect. PERT does not increase mucus production in the airways; it is focused on the digestive system.

Incorrect. Increasing sugary snacks is not advisable for CF patients. They need a balanced diet to meet their nutritional needs.

Incorrect. Deep breathing exercises should be encouraged regularly, not just during illness, to maintain airway clearance and lung function.

Correct. CF is characterized by thick, sticky mucus that can lead to chronic respiratory infections, making this a key clinical manifestation to monitor.

Incorrect. Vigorous exercise is beneficial for CF patients but should not replace specific airway clearance techniques.

Correct. Regular follow-up appointments with the CF care team are crucial to monitor the child's health and adjust the treatment plan as needed.
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