Cystic Fibrosis

Which of the following signs and symptoms are expected in a patient with untreated cystic fibrosis? (Select ALL that apply.)

A. Coughing and shortness of breath C. Poor weight gain and growth despite adequate food intake D. Steatorrhea The classic symptoms of untreated cystic fibrosis include: salty-tasting skin and poor weight gain and growth despite adequate food intake. Most patients will have steatorrhea (fatty stools) from deficient pancreatic enzyme production. Cough, shortness of breath, thick mucus and frequent lung infections are common. Epistaxis is the clinical term for a nose bleed, which is not associated with CF.

Which of the following is a true statement about dornase alfa?

A. Doses should not be mixed with any other medications in the nebulizer Which of the following is a true statement about dornase alfa? Dornase alfa (Pulmozyme) comes in ampules which are given in a nebulizer once daily. The ampules are stored in the refrigerator and must be protected from light. This medication should not be mixed with any other drug in the nebulizer.

DB is newly diagnosed with cystic fibrosis. His family is being counseled on the use of chest percussion, inhaled bronchodilators and inhaled antibiotics. What is the proper order in which he should receive these therapies?

A. Inhaled bronchodilators, followed by chest percussion, followed by inhaled antibiotics It is important to counsel patients on the order of chest percussion (or chest physiotherapy) in relation to the medications. Chest physiotherapy is typically performed by a physical or respiratory therapist. It involves physical percussion, vibration and deep breathing/coughing techniques to improve breathing.

Which of the following pathogen/s is/are most likely to cause lung infections in a 3-year-old child with CF?

A. Staphylococcus aureus and Haemophilus influenzae The most common organisms early in the disease are Staphylococcus aureus and Haemophilus influenzae.

When starting pancreatic enzyme treatment, the dose is increased as needed based on which of the following?

A. Stool consistency The dose is individualized for each patient and is adjusted every 3-4 days until stools are normalized.

Which of the following are correct counseling points for the PEP regimen? (Select ALL that apply.)

A. Take half of a mealtime dose with snacks B. Do not crush or chew the capsules D. Drink plenty of water with each dose E. High-fat meals may require higher doses Pancreatic enzymes should be taken with snacks at half the mealtime dose. Capsules should be swallowed whole and taken with plenty of water. Capsules may be opened and sprinkled on soft foods such as applesauce, pureed bananas or pears and consumed right away

(same as above) Which of the following are correct counseling points for the PEP regimen? (Select ALL that apply.)

A. Take half of a mealtime dose with snacks B. Do not crush or chew the capsules D. Drink plenty of water with each dose E. High-fat meals may require higher doses Pancreatic enzymes should be taken with snacks at half the mealtime dose. Capsules should be swallowed whole and taken with plenty of water. Capsules may be opened and sprinkled on soft foods such as applesauce, pureed bananas or pears and consumed right away. A high-fat content meal may require a higher dose of pancreatic enzymes to break down the additional fat consumed.

Which of the following are correct statements concerning pancreatic enzymes (pancrelipase products)? (Select ALL that apply.)

A. The enzymes work in the gut to break down fat, starches and protein for better absorption B. The enzymes should be stored in a dry location E. The products contain lipase, amylase and protease Pancreatic enzymes work in the gut to help break down fat, starch and protein. Pancrelipase products contain the enzymes lipase, amylase and protease. The enzymes should be protected from moisture by being kept in an environment that is not humid.

What is the rationale for using nebulized hypertonic saline (HyperSal) in cystic fibrosis?

A. To mobilize the mucus Hypertonic saline is administered via a nebulizer to mobilize mucus for improved airway clearance. Hypertonic saline is a high-risk medication that can be fatal if administered too rapidly via IV infusion (refer to Acute & Critical Care Medicine and Medication Safety & Quality Improvement chapters).

DP is a 28-year-old male with CF diagnosed at one year of age. He presents to the pulmonologist for evaluation of his chronic lung infections. Which of the following is/are the most likely pathogen/s?

B. Pseudomonas aeruginosa In adolescents and adults, the most prevalent organism is Pseudomonas aeruginosa for both acute and chronic pulmonary infections.

What is the purpose of performing a sweat test?

B. To confirm the diagnosis of CF A sweat test is used to confirm the diagnosis of CF. The sweat test measures the amount of salt in the sweat, which is high in patients with CF.

Patients with cystic fibrosis are at risk of the following vitamin deficiencies:

E. Vitamins A, D, E, and K Patients with cystic fibrosis have trouble absorbing proteins and fats, including the fat-soluble vitamins A, D, E and K, often making vitamin supplementation necessary. A high-fat and calorically-dense diet is recommended to assist with adequate growth, to meet energy needs and prolong survival. Many patients with CF will eventually require insulin for the treatment of CF-related diabetes mellitus.

Which of the following is an inhaled antibiotic used in cystic fibrosis?

B. Aztreonam Cayston is the brand name for aztreonam inhalation solution. Aztreonam IV is branded as Azactam.

Cystic fibrosis (CF) is a condition in which mucus production negatively affects which of the following organs? (Select ALL that apply.)

B. Lungs D. Pancreas E. Intestines Patients with CF produce thick mucus that affects the lungs, pancreas, intestines and liver.

A pediatric nurse is treating a patient with cystic fibrosis. The patient needs to be taken to radiology for a lung CT this afternoon. She would like to give the next dose of Cayston as early as possible to accommodate the scheduled imaging. What is the earliest time that she can give the next dose?

C. 1200 TID dosing of aztreonam should be scheduled as close to Q8H as possible. This provides optimal bacterial killing around the clock, while minimizing the time when the antibiotic concentration is low (which can worsen resistance). Q8H dosing requires waking the patient up in the middle of the night, so it is usually more convenient to give doses during waking hours (as we see in this case). The doses must be at least 4 hours apart to provide adequate drug concentrations throughout the day.

Which medication is used to open the airway in CF before administering other medications?

C. Albuterol Albuterol is a short-acting beta-2 agonist (bronchodilator) which is administered before all other inhaled medications in CF. Albuterol opens the airways, allowing for improved delivery of the remaining medications.

Which of BG's home medications required genotype testing prior to use?

C. Lumacaftor/ ivacaftor Lumacaftor/ivacaftor (Orkambi) is indicated for the homogenous F508del mutation only. Since each CFTR modulator is approved for very specific mutations, genotype testing must be performed to ensure the appropriate drug is initiated. The genotype testing would be done once before the medication was initially prescribed.

Which of BG's home medications required genotype testing prior to use?

C. Lumacaftor/ivacaftor Lumacaftor/ivacaftor (Orkambi) is indicated for the homogenous F508del mutation only. Since each CFTR modulator is approved for very specific mutations, genotype testing must be performed to ensure the appropriate drug is initiated. The genotype testing would be done once before the medication was initially prescribed.

BG is a 15-year-old female with CF presenting to her pulmonologist complaining of SOB and increased albuterol use over the past few weeks. She states that she has been compliant with all of her medications. Home medications: TOBI 300 mg via nebulizer Q12H Pulmozyme 2.5 mg via nebulizer daily Albuterol 2.5 mg via nebulizer Q4-6H PRN Hypertonic saline 4 mL via nebulizer Q12H Zenpep 10,000 units of lipase/kg/day PO as directed Lumacaftor/ivacaftor (Orkambi) 2 tablets PO Q12H Lansoprazole 15 mg PO daily Which of the following is the correct way to cycle the inhaled antibiotic?

D. 4 weeks on followed by 4 weeks off Tobramycin (TOBI) should be given every 12 hours for 28 days (4 weeks), followed by 28 days off cycle.

(same as above) In what order should the inhaled therapies and chest physiotherapy be given? Rank the following from first to last. (Left-click to drag and order the answer options; the first therapy must be placed in the top box. ALL options must be used.)

D. Albuterol C. Hypertonic saline E. Pulmozyme B. Chest physiotherapy A. TOBI Albuterol should be given first to open up the airway for improved delivery of other medications. The antibiotic should always be given last to maximize delivery to the lungs.

What is the TOBI Podhaler?

E. The inhalation device used with TOBI capsules The TOBI Podhaler device comes in a pack with capsules containing tobramycin inhalation powder for patients with CF. It is similar to the Spiriva Handihaler and other dry powder inhalers that use a capsule for asthma/COPD.

BG is a 15-year-old female with CF presenting to her pulmonologist complaining of SOB and increased albuterol use over the past few weeks. She states that she has been compliant with all of her medications. Home medications: TOBI 300 mg via nebulizer Q12H Pulmozyme 2.5 mg via nebulizer daily Albuterol 2.5 mg via nebulizer Q4-6H PRN Hypertonic saline 4 mL via nebulizer Q12H Zenpep 10,000 units of lipase/kg/day PO as directed Lumacaftor/ivacaftor (Orkambi) 2 tablets PO Q12H Lansoprazole 15 mg PO daily In what order should the inhaled therapies and chest physiotherapy be given? Rank the following from first to last. (Left-click to drag and order the answer options; the first therapy must be placed in the top box. ALL options must be used.)

D. Albuterol C. Hypertonic saline E. Pulmozyme B. Chest physiotherapy A. TOBI Albuterol should be given first to open up the airway for improved delivery of other medications. The antibiotic should always be given last to maximize delivery to the lungs.

What causes cystic fibrosis?

D. Mutation in the CFTR gene The name cystic fibrosis refers to the characteristic scarring (fibrosis) and cyst formation that occurs within the pancreas. It is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (referred to as "CFTR"). CFTR regulates the movement of chloride, bicarbonate and sodium ions across epithelial membranes.

(same as above) BG is admitted to the hospital for IV antibiotics. The pharmacist realizes that the hospital does not have Zenpep on formulary and would like to substitute with Creon. What should the pharmacist do?

D. Substitute at the prescriber's discretion to the most equivalent dose of Creon. Per the FDA, PEPs are not interchangeable. Hospitals usually do not stock all of the commercially available products, so a new prescription order is required in order to switch the patient during hospital admission. When switching a patient to another pancreatic enzyme product, consider starting with a similar amount of lipase enzyme, then adjust the dose based on the patient's response. It would not be appropriate to simply omit the PEP for a patient with CF during an inpatient hospitalization.

BG is admitted to the hospital for IV antibiotics. The pharmacist realizes that the hospital does not have Zenpep on formulary and would like to substitute with Creon. What should the pharmacist do?

D. Substitute at the prescriber's discretion to the most equivalent dose of Creon. Per the FDA, PEPs are not interchangeable. Hospitals usually do not stock all of the commercially available products, so a new prescription order is required in order to switch the patient during hospital admission. When switching a patient to another pancreatic enzyme product, consider starting with a similar amount of lipase enzyme, then adjust the dose based on the patient's response. It would not be appropriate to simply omit the PEP for a patient with CF during an inpatient hospitalization.

(same as above) How should BG be counseled to take her PEP?

D. Take a dose at the beginning of each meal The entire dose of pancreatic enzymes should be taken at the beginning of each meal. The enzymes are needed in the gut to breakdown and absorb food and nutrients.

How should BG be counseled to take her PEP?

D. Take a dose at the beginning of each meal. The entire dose of pancreatic enzymes should be taken at the beginning of each meal. The enzymes are needed in the gut to breakdown and absorb food and nutrients.

To prevent Viokace tablets from breaking down in the stomach, they must be:

D. Taken with a proton pump inhibitor Viokace is a non-enteric coated tablet. To prevent destruction in the stomach, it must be taken with a PPI.

Why are nebulized medications used frequently in cystic fibrosis? (Select ALL that apply.)

D. To minimize the risk of systemic toxicity E. To deliver the medication directly to the site of action Nebulized medications deliver the dose directly to the lungs. Inhaled medications have minimal systemic absorption, which reduces the risk of systemic toxicity. Nebulized antibiotics are generally more expensive and less convenient than IV administration of the same antibiotic.

Why is Pulmozyme used in cystic fibrosis?

D. To thin the mucus Dornase alfa is an enzyme that decreases (thins) mucus viscosity, promoting airway clearance.

Which of the following patients is most likely to benefit from Zenpep?

E. A patient who has had a pancreatectomy Zenpep is a pancreatic enzyme replacement product. Patients who have had a pancreatectomy (removal of the pancreas) would not be able to produce pancreatic enzymes on their own.

Pancreatic enzyme products (PEPs) are formulated to dissolve in the:

E. Basic pH of the duodenum Pancreatic enzyme products (PEPs) are formulated to dissolve in the more basic pH of the duodenum.