Question 1

You are examining a 12-year-old with cystic fibrosis. His resting vitals are HR = 90 bpm; RR = 20 bpm; and SpO₂ = 98%. You start your exercise testing. As the test continues, the child is walking on the treadmill at 2.5 mph 2% grade for 5 min. His vital signs are as follows: HR = 120 bpm, RR = 22 bpm, SpO₂, 90%; and an RPE of 14. You would

A) continue the test with the same mph and grade.
B) increase the mph and grade.
C) decrease the grade but keep the mph the same.
D) stop the test and monitor vitals over the next few minutes.
E) stop the test and call a code.

D
Explanation: You would stop the test as the SpO₂ is dropping; you would want to stop and confirm the SpO₂ and then call his nurse and report what your examination found.

Accepted Answer

You would stop the test as the SpO₂ is dropping; you would want to stop and confirm the SpO₂ and then call his nurse and report what your examination found.

Question 2

You are evaluating a 16-year-old with cystic fibrosis who needs an independent method of postural drainage with percussion and vibration (PDPV). The most appropriate method for the patient is&#10;A) percussor.&#10;B) positive expiratory pressure and flutter.&#10;C) a vest.&#10;D) PDPV from his friends.

Accepted Answer

A vest gives a teenager the most comprehensive method of clearing all lobes of the lung in the most independent way possible.

Question 3

You are doing an examination on a 17-year-old with cystic fibrosis who confides in you that she is smoking on a daily basis. You should&#10;A) report it to cystic fibrosis clinic practitioners and document it in her medical record.&#10;B) tell her &#34;You are not helping the situation.&#34;&#10;C) call her parents right away and tell them they have a problem child.&#10;D) ignore the statement; all teens talk this way.

Accepted Answer

Smoking has a direct impact on pulmonary health and needs to be addressed because it poses a serious health risk with potential for long-term addiction. Smoking cessation requires a team management approach from health practitioners that are in more frequent contact with the patient than the physical therapist.

Question 4

A child can have cystic fibrosis and have no pulmonary symptoms.

Accepted Answer

The gastrointestinal symptoms, which may include poor weight gain, intolerance of fatty foods, and floating stools, can occur due to pancreatic insufficiency without any pulmonary signs.

Question 5

The median predicted life expectancy for patients with cystic fibrosis is approximately&#10;A) 18 years.&#10;B) 28 years.&#10;C) 38 years.&#10;D) 48 years.

Accepted Answer

The median predicted life expectancy for patients with cystic fibrosis has increased from 32 years in the year 2000 to 37.42 years in the year 2008.

Question 6

During a meeting with a family regarding their newborn son, you review postural drainage with percussion and vibration (PDPV). They state that they do not believe that PDPV is important at this time. You would

A) tell them that if they don't do PDPV, their son may die sooner.
B) request a Department of Youth and Family Services consult.
C) review why PDPV is important and how it could impact the health and functioning of their son's pulmonary system now and in the future.
D) call in the nurse and ask her to tell the family they must comply.

Accepted Answer

It is important to clearly state the reasons the family should perform PDPV and how it will impact their child's future pulmonary health. A new diagnosis of cystic fibrosis is very stressful to families and they need some time to adjust and balance the new health care responsibilities with their other life demands.

Question 7

During your examination of a 14-year-old with cystic fibrosis, you note moderate clubbing throughout all digits. This clubbing is caused by&#10;A) acute hypoxemia.&#10;B) chronic hypoxemia.&#10;C) lack of performance of fine motor skills.&#10;D) medication side effects.

Accepted Answer

Clubbing is thought to be caused by chronic hypoxia and the body's attempt at gaining more oxygen by proliferating blood vessels in the extremities.

Question 8

You are examining a 2-month-old with cystic fibrosis. The family is meeting with you to learn postural drainage percussion and vibration (PDPV). You would teach them all the PDPV positions except&#10;A) left side lying.&#10;B) prone.&#10;C) Trendelenberg.&#10;D) right side lying.

Accepted Answer

Trendelenberg is to be avoided in a 2-month-old due to the likelihood this will increase gastroesophagel reflux.

Question 9

Cystic fibrosis involves the exocrine mucous glands and typically affects the&#10;A) genitourinary tract.&#10;B) pancreas.&#10;C) sweat glands.&#10;D) All of the above

Accepted Answer

Cystic fibrosis involves the exocrine mucous glands and typically affects the lungs, liver, pancreas, gastrointestinal tracts, sweat glands, and the genitourinary tracts. The severity of the disease and the organs affected depends largely on the specific type of genetic mutation involved.

Question 10

An 8-year-old child with cystic fibrosis presents to the clinic with a 1-wk history of persistent cough, increased sputum production, increased work of breathing, and weight loss. What steps should be taken next?

A) He should be sent to the emergency room of a local hospital for critical care.
B) He should most likely be admitted to the hospital for treatment of a pulmonary exacerbation.
C) He should be sent home on extra multivitamins and be seen for his next routine clinic visit in 6 months.
D) He should be sent home, his pulmonary hygiene program should be stopped, and his parents/caregivers should be instructed to focus on his nutrition for a few weeks.

Accepted Answer

These are signs of a pulmonary exacerbation, which would most likely need coordinated care along with antibiotics. It is not an emergency situation, but does call for medical intervention.

Deck 20: Cystic Fibrosis