The parents of a 3-month old infant with cystic fibrosis (CF) want to know how their child got this disease, because no one in either of their families has CF. What is the nurse's best response based on the understanding of CF?

Both parents are carrier of the CF gene

The inheritance pattern is multifactorial

The result is probably a genetic mutation

Only one parent carries the CF gene

Answer and Explanation

The Correct Answer is A

A. Both parents are carriers of the CF gene: Cystic fibrosis follows an autosomal recessive inheritance pattern, meaning both parents must carry one copy of the defective gene, even if they do not have the disease themselves. The child inherits one copy of the defective gene from each parent to develop CF.

B. The inheritance pattern is multifactorial: CF is not a multifactorial disease; it follows a clear recessive genetic inheritance pattern.

C. The result is probably a genetic mutation: CF is caused by a genetic mutation, but this mutation is inherited from both parents. It is not usually a spontaneous mutation unless it occurs in the parental genetic material, which is rare.

D. Only one parent carries the CF gene: For the child to inherit CF, both parents must be carriers of the gene. If only one parent carries the gene, the child would be a carrier, not affected by the disease.

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Correct Answer is C

Explanation

A. Place the child in a sitting position and tilt her head back: Tilting the head back risks blood aspiration or swallowing, which can cause nausea or vomiting.

B. Apply ice at the opening of the nares for 5 minutes and then re-check for bleeding: Ice may help, but direct pressure is the first-line intervention.

C. Have the child sit with her head tilted forward and hold pressure on her nose for 10 min: This is the appropriate action to stop bleeding and prevent blood from entering the airway or stomach.

D. Place the child in a supine position with a pillow under her head: This position is unsafe as it can lead to blood pooling in the back of the throat.

A. Upright B. Side-lying C. Supine D. Prone

Correct Answer is A

Explanation

A. Upright: In an upright position, gravity helps expand the lungs, allowing for maximal ventilation and improved oxygenation. This position helps the diaphragm move downward and increases lung volume, making it easier to breathe.

B. Side-lying: Side-lying positions may be helpful in certain situations, such as in cases of aspiration pneumonia, but they do not provide the best lung expansion for respiratory failure.

C. Supine: Lying flat on the back can increase the work of breathing and make it harder for the lungs to fully expand, especially in a child with respiratory distress.

D. Prone: The prone position is sometimes used in cases of acute respiratory distress syndrome (ARDS) in adults, but it is not commonly recommended for children with pneumonia unless specifically ordered by the healthcare provider.

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The parents of a 3-month old infant with cystic fibrosis (CF) want to know how their child got this disease, because no one in either of their families has CF. What is the nurse's best response based on the understanding of CF?

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