AIRWAYS UNDER PRESSURE: NAVIGATING CYSTIC FIBROSIS PULMONARY CARE
Faculty:
L. Austin Fredrickson, MD, FACP
L. Austin Fredrickson is an Associate Professor of Internal Medicine at Northeast Ohio Medical University, where he serves as core faculty and teaches diagnostics, therapeutics, clinical skills, and health humanities. He is board-certified in general internal medicine and practices rural primary care.
Sandra Rogers, MD
Sandra Rogers, MD, is a primary care physician in Texas. She is board-certified through the American Board of Family Medicine and the American Board of Internal Medicine.
Jennifer Salvon, RPh
Jennifer Salvon, RPh, is a clinical pharmacist at Mercy Medical Center and a freelance medical writer at Salvon Scientific in Massachusetts. Her career includes practice in hospital, retail, managed care, academic, and clinical research settings. As a lifelong learner, Jen enjoys researching and writing to educate herself and others.
Pamela Sardo, PharmD, BS
Pamela Sardo, PharmD, BS, is a freelance medical writer and licensed pharmacist. She is the founder and principal at Sardo Solutions in Texas. Pam received her BS from the University of Connecticut and her PharmD from the University of Rhode Island. Pam’s career spans many years in retail, clinics, hospitals, long-term care, Veterans Affairs, and managed health care responsibilities across a broad range of therapeutic classes and disease states.
Abstract
Cystic fibrosis (CF) is classified as a rare disease by the National Organization of Rare Disorders, yet it is also one of the most diagnosed genetic disorders. In the US, approximately 30,000 people live with CF, and 1,000 new cases are diagnosed each year. Cystic fibrosis primarily affects the lungs and digestive system, but its impact extends throughout the body. Pulmonary complications, including progressive obstructive lung disease, sinusitis, and infections, contribute to significant disease burden. While CF is known to reduce life expectancy, advancements in early detection through newborn screening, adherence to evidence-based pulmonary care guidelines, and the establishment of specialized CF care centers have steadily improved survival rates. A multidisciplinary team in CF care typically includes physicians, nurses, dietitians, physiotherapists, psychologists, and pharmacists, all working together to manage complex patient needs. The introduction of new treatments and ongoing research is expected to yield ongoing improvements in health outcomes and quality of life for individuals living with cystic fibrosis.
Accreditation Statements
In support of improving patient care, RxCe.com LLC is jointly accredited by the Accreditation CouncilTM for Continuing Medical Education (ACCME®), the Accreditation Council for Pharmacy Education (ACPE®), and the American Nurses Credentialing Center (ANCC®), to provide continuing education for the healthcare team.
Joint Universal Activity Number: The Joint Accreditation Universal Activity Numbers assigned to this activity are as follows:
Pharmacists: JA4008424-0000-26-098-H01-P
Pharmacy Technicians: JA4008424-0000-26-098-H01-T
Credits: 2 contact hour(s) (0.2 CEU(s)) of continuing education credit.
Credit Types:
Pharmacy - 2 Credits
Type of Activity: Application
Media: Computer-Based Training (i.e., online courses)
Estimated time to complete activity: 2 contact hour(s) (0.2 CEU(s)), including Activity Pre-Test, Post-Test, and Activity Evaluation.
Release Date: June 22, 2026 Expiration Date: October 17, 2028
Target Audience: This educational activity is for Pharmacists and Pharmacy Technicians
How to Earn Credit: From June 22, 2026, through October 17, 2028, participants must:
Read the “learning objectives” and “author and planning team disclosures;”
Take the “Educational Activity Pre-Test;”
Study the section entitled “Educational Activity;” and
Complete the Educational Activity Post-Test and Activity Evaluation. The Educational Activity Post-Test will be graded automatically. Following successful completion of the Educational Activity Post-Test with a score of 70% or higher, a statement of participation will be made available immediately. (No partial credit will be given.)
CE Credits: Credits for this course will be uploaded to CPE Monitor® for pharmacists and pharmacy technicians.
Statement of Need
Cystic fibrosis (CF) is a complex, inherited genetic disorder that primarily affects the lungs and digestive system, but its impact extends to multiple organs throughout the body. A multidisciplinary team is more effective in CF care. Team members include physicians, nurses, dietitians, physiotherapists, psychologists, and pharmacists. Team members need to know how to work together to manage the complex needs of CF patients.
Learning Objectives: Upon completion of this educational activity, participants should be able to:
Describe the genetic mutations and molecular mechanisms underlying cystic fibrosis (CF)
Identify diagnostic methods and clinical indicators used to diagnose CF
Recognize complications associated with CF
Discuss the current recommendations for long-term lung health in CF patients
Disclosures
The following individuals were involved in planning, developing, and/or authoring this activity: L. Austin Fredrickson, MD, FACP; Sandra Rogers, MD; Jennifer Salvon, RPh; and Pamela Sardo, PharmD, BS. None of the individuals involved in developing this activity has a conflict of interest or financial relationships related to the subject matter. There are no financial relationships or commercial or financial support relevant to this activity to report or disclose by RxCe.com or any of the individuals involved in the development of this activity. © RxCe.com LLC 2026: All rights reserved. No reproduction of all or part of any content herein is allowed without the prior, written permission of RxCe.com LLC.
Educational Activity Pre-Test
Cystic fibrosis mainly affects which system of the body?
Nervous system
Skeletal system
Respiratory system
Urinary system
Which symptoms in a baby or young child might help identify CF?
Pale stools and ear infections
Skin that appears jaundiced and forced vital capacity (FVC) ≥ 90%
Frequent wheezing and no mucus
Chronic cough and thick phlegm
Cystic fibrosis patients with _____________ should not fly on a plane, perform spirometry, or lift weights for the 2-week period after the pneumothorax has resolved.
hemoptysis
chronic rhinosinusitis
bacterial infection
pneumothorax
Educational Activity
Airways Under Pressure: Navigating Cystic Fibrosis Pulmonary Care
Introduction
Cystic fibrosis (CF) is a complex, inherited genetic disorder that primarily affects the lungs and digestive system, but its impact extends to multiple organs throughout the body. Thick, sticky mucus resulting from the faulty CFTR gene leads to chronic respiratory problems, frequent infections, and significant digestive difficulties, making CF a lifelong, progressive disease that varies in severity among patients. Advancements in treatments and multidisciplinary care have improved outcomes, enabling many individuals to live longer and healthier lives, though respiratory failure remains a leading cause of mortality.
Understanding the Burden and Biology of Cystic Fibrosis
The National Organization for Rare Diseases (NORD) designates CF as a rare disease.1 A rare disease is defined by its low prevalence within the population. In the US, according to the Food and Drug Administration (FDA) and the Orphan Drug Act, a rare disease is defined as a condition affecting fewer than 200,000 people.2
Approximately 30,000 people in the United States live with CF, with 1,000 new cases diagnosed each year.3,4 Occurring across all racial and ethnic groups, CF is most common among Caucasians, with a frequency of 1 in 2,500.5,6 One in twenty-five people of European descent is a healthy carrier of a gene mutation that may cause CF.7
Data show an incidence of 1 in 8,000 among Hispanic individuals, 1 in 15,000 among people of African descent, and 1 in 35,000 among Asians.6 Minority groups are reportedly underrepresented in clinical trials and may suffer poorer outcomes than their Caucasian counterparts.8
Cystic fibrosis is a genetic disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, located on chromosome 7q31.2.7 The CFTR gene encodes a protein that functions as a chloride and bicarbonate channel in epithelial cells, playing a critical role in regulating the movement of salt and water across cell membranes.7,9
Cystic fibrosis follows an autosomal recessive pattern. To develop the disease, an individual must inherit two defective copies of the CFTR gene, one from each parent.7 Individuals with only one mutation are carriers and typically do not show clinical symptoms.7,9
Defective or absent CFTR protein impairs chloride and bicarbonate transport, leading to dehydrated, thick, and sticky mucus secretions.7,9 This underlies CF's characteristic pulmonary, pancreatic, gastrointestinal, and reproductive complications. The disease process causes thick mucus to accumulate and clog the airways.7,9 The mucus retention leads to chronic lung infections and damaging airway inflammation.7,9
More than 2,000 CFTR mutations have been identified, but not all cause disease.7 These mutations are grouped into five functional classes, depending on how they disrupt CFTR protein production or function.7,8
Table 1 below outlines the CFTR classes.
Table 1
CTFR Mutation Classification7,9
| Class | Defect | Effect | Patients | Mutation Examples |
| 1 | No production of CFTR protein | Severe | 22% | G542X W1282X R553X |
| 2 | CFTR protein is produced, but misfolded, and does not reach the cell surface | Severe | 88% | F508del N1303K 1507del |
| 3 | CFTR protein is produced, reaches the cell surface, but fails to function properly | Severe | 6% | G551D S549N |
| 4 | CFTR protein is produced, and chloride flow through the channel is reduced | Mild to moderate | 6% | D1152H R347P R117H |
| 5 | CFTR protein is produced in reduced amounts | Mild to moderate | 5% | A455E |
Life expectancy for people with CF was historically low but has improved significantly with recent medical advances.10 Cystic fibrosis was first described in 1938, with a median survival age of 4-5 years.10 The establishment of CF Centers in the 1950s led to improved management and increased median survival to the mid-20s.10 Key interventions included aggressive treatment of complications, improved nutrition, and better management of lung disease.10
Based on US data from 2019, the Cystic Fibrosis Foundation Registry Report predicts a median survival age of a child born that year with CF to be 48.4 years.10 The median age of death among CF decedents continued to increase, indicating improved survival.11 The leading causes of mortality in CF remain respiratory failure, complications from transplantation, and CF-related liver disease.10 Continued improvements in comprehensive care programs mean CF is often a manageable chronic condition for many patients.10 Improvements in comprehensive care have also led to a decline in the number of transplantations in CF patients. This is important since complications from transplantation are among the leading causes of mortality in CF.10
Screening and Diagnosis
Diagnosis of CF involves clinical assessment, family history, and laboratory tests, beginning with newborn screening, followed by sweat chloride analysis and confirmatory genetic testing. In the US, newborn screening (NBS) for CF is routine, performed shortly after birth using a blood sample obtained by heel prick. The blood is analyzed for elevated levels of immunoreactive trypsinogen (IRT), an enzyme released by the pancreas.12,13 High IRT levels can suggest CF but are not diagnostic, as levels may also be elevated due to prematurity or birth stress. Screening is considered positive if the IRT level remains elevated between 7 and 14 days of life.3
The sweat chloride test is the gold standard for diagnosing CF, measuring chloride levels in sweat.12,13 Cystic fibrosis causes elevated sweat chloride due to faulty chloride channels. Sweat is induced using transdermal pilocarpine. It is then collected and analyzed. A result of ≥ 60 mmol/L indicates CF.12,13 If sweat testing is inconclusive, genetic testing for CFTR gene mutations is performed.
Cystic fibrosis can be diagnosed through a positive newborn screening and clinical features like chronic sinus or lung disease, or via family history and signs of CFTR dysfunction (such as high sweat chloride levels).14 Early diagnosis of an asymptomatic infant with CF allows timely interventions to slow lung disease progression and the provision of psychosocial support to families.3 At least 64% of new CF diagnoses in the US now occur in asymptomatic or minimally symptomatic infants.15 While screening has many benefits, it may also lead to increased medical interventions, financial burdens from CF therapies, risk of exposure to respiratory pathogens during clinic visits, potential side effects from treatments, and caregiver anxiety.3
Spirometry is a simple yet essential breathing test used to manage CF and assess lung function by measuring the amount and speed of air exhaled from the lungs. Patients breathe into a spirometer to obtain key measurements, including forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), and the FEV1/FVC ratio.12 These values help identify obstructive or restrictive patterns commonly seen in CF. This test is crucial for diagnosing, monitoring disease progression, and detecting exacerbations.12 Clinicians closely watch changes in spirometry values to determine the impact of therapies and the need for interventions or medication adjustments.
Clinical Presentation
The clinical presentation of CF is diverse due to the numerous CFTR variants and the wide range of protein dysfunction. Respiratory symptoms are common and include a persistent, often productive cough with thick mucus, wheezing, shortness of breath, and exercise intolerance.3,12,13 Patients are prone to frequent and recurrent lung infections such as pneumonia and bronchitis. Many patients experience chronic sinusitis or nasal polyps, often accompanied by inflamed or stuffy nasal passages.12,13
Gastrointestinal involvement typically manifests as greasy, foul-smelling stools (steatorrhea), failure to thrive or poor weight gain despite a good appetite, and intestinal blockage, especially meconium ileus in newborns.12,13 Severe constipation, abdominal pain, bloating, and rectal prolapse are also possible, largely due to malabsorption and pancreatic insufficiency.
Children may display delayed growth and development, while adults could present with milder or atypical forms, such as recurrent pancreatitis, pneumonia, or infertility.12,13 Additional features of CF include salty-tasting skin, musculoskeletal problems like osteoporosis, clubbing of the fingers, and electrolyte disturbances such as hyponatremia and metabolic alkalosis.12,13 Liver disease, including biliary cirrhosis, may also develop in some individuals.12,13
Cystic Fibrosis Management
Management of CF is comprehensive and multidisciplinary, targeting symptoms and underlying complications to improve quality of life and prolong survival. Cystic fibrosis’s effects on multiple organ systems necessitate a team that includes pulmonologists, gastroenterologists, infectious disease specialists, dietitians, nurses, and pharmacists.16 Core pillars of CF pulmonary management include the following:12,13
Airway clearance techniques to loosen and remove the thick mucus from the lungs
Treatment and prophylaxis of chronic pulmonary infections
Reduction of chronic airway inflammation
Treatment of pulmonary complications
Medications are essential in CF management. CFTR modulators directly address the underlying protein defect in patients with eligible mutations. Antibiotics treat and help prevent respiratory infections. Mucus-thinning agents, bronchodilators, and anti-inflammatory drugs target thick mucus secretions.
CFTR Modular Therapies
CFTR modulator therapy is the first targeted CF treatment approach to address the underlying protein defect caused by the CFTR mutation. Rather than managing symptoms, CFTR modulators directly improve the function of the defective CFTR protein.12,13 There are two types of CFTR modulators currently available: potentiators and correctors. Potentiators improve CFTR function at the cell surface by increasing channel opening, particularly in gating mutations such as G551D.17 Ivacaftor and deutivacaftor are available FDA-approved potentiators.18,20,21 A triple therapy combining elexacaftor-tezacaftor-ivacaftor (Trikafta®) was approved by the FDA in 2019.19,22
Correctors help misfolded CFTR proteins reach the cell surface and are mainly helpful for the F508del mutation. When combined with potentiators, they are effective for a larger number of CF patients. Available correctors include lumacaftor, tezacaftor, elexacaftor, and vanzacaftor.20-24 Table 2 below lists available CFTR modulator therapies.
Table 2
CFTR Modulator Therapies20-24
| Drug | Type | Dosing | Treatment considerations |
Ivacaftor (Kalydeco®) | Potentiator | · ≥ 6 yrs: 150 mg PO q12h with fat-containing food · Pediatric granule packets available for ≥ 4 months | · Monitor LFTs · Assess vision (cataract risk in pediatrics) · Avoid strong CYP3A inducers · Adjust for hepatic impairment |
Lumacaftor/ Ivacaftor (Orkambi®) | Corrector & potentiator | · ≥ 6 yrs: lumacaftor 400 mg/ ivacaftor 250 mg PO q12h with fat-containing food | · High rate of drug–drug interactions (strong CYP3A inducer) · May cause respiratory adverse effects · Monitor BP and LFTs |
Tezacaftor/ Ivacaftor (Symdeko®) | Corrector & potentiator | · ≥ 6 yrs: tezacaftor 100 mg/ Ivacaftor 150 mg PO AM + ivacaftor 150 mg PO PM with fat-containing food. | · Better tolerated than lumacaftor/ivacaftor combo · Monitor LFTs and vision in pediatrics · Fewer DDIs but still metabolized by CYP3A |
Elexacaftor/ Tezacaftor/ Ivacaftor (Trikafta®) | Triple therapy: 2 correctors & 1 potentiator | ≥ 12 yrs: · 2 orange tablets (elexacaftor 100 mg/ tezacaftor 50 mg/ ivacaftor 75 mg each) PO in AM · 1 blue tablet (ivacaftor 150 mg) PO in PM · With fat-containing food · Dosing adjustments available for ages ≥ 2 yrs. | · Highly effective · Monitor LFTs · Assess for CYP3A interactions · Check vision in pediatrics · Watch for rash (esp. in adolescent females) · Dose adjust in patients with hepatic impairment |
Vanzacaftor/ Tezacaftor/ Deutivacaftor (Alyftrek®) | Triple therapy: 2 correctors & 1 potentiator | ≥ 12 yrs: · 2 tablets once daily · Each tablet = 10 mg vanzaacaftor, 50 mg tezacaftor, 125 mg deutivacaftor Age 6 to < 12 years and weight ≥ 40 kg: same as adult Age 6 to < 12 years, weight < 40 kg: 3 tablets | · Take at the same time every day · Do not crush, split, or chew tablets · Swallow tablets whole · Take with fat-containing food |
CFTR modulator therapies are linked to measurable improvements in several clinical indicators. They support lung function by increasing forced expiratory volume (FEV1), decreasing pulmonary exacerbations, and alleviating respiratory symptoms.12,25 Nutritional benefits include an increase in body mass index (BMI) and improved pancreatic function. Reduced hospital stays and improved long-term prognoses result in a better quality of life.25 Improvements in sweat chloride levels reflect improved CFTR protein function.25
Early treatment can lead to better lung and pancreatic outcomes and improved nutritional status, especially in children.25 Further data on their long-term effectiveness in everyday clinical practice are still being collected.
Only people with specific CFTR mutations are eligible for modulator therapy, necessitating genetic testing before therapy initiation.12 CFTR modulator research is ongoing. Some patients may not tolerate these drugs well or may have suboptimal responses, highlighting the need for personalized treatment plans.
CFTR modulator therapy represents a major shift in CF care by addressing the underlying molecular defect rather than just managing symptoms. Advances in combination therapies are steadily broadening patient eligibility and enhancing the benefits these treatments offer.
Therapies to Manage Lung Health
Long-Term Clearance of Airway Secretions
Cystic fibrosis management includes daily airway clearance techniques (ACTs) to mobilize and remove airway secretions. Removing airway mucus clears irritants, decreases the respiratory bacterial load, improves gas exchange, and reduces airway obstruction.3,26
Airway clearance techniques include the following:3,12,13,26
Chest physical therapy
Mechanical devices (e.g., vibrating vests)
Active cycle breathing techniques
Positive expiratory pressure (PEP) devices
Oscillatory devices
Regular aerobic exercise
Dornase alpha is a recombinant human deoxyribonuclease. Inhaled as an aerosol, it enzymatically cleaves extracellular DNA released from degenerating neutrophils in airway mucus.27,28 This DNA contributes to the increased viscosity and tenacity of sputum in patients with CF. Dornase alpha treatment significantly reduces mucus viscosity, enhancing clearance by ciliary and cough mechanisms.27,28 Pulmonologists use FVC measurements when considering dornase alpha. In patients with an FVC ≥ 40% of predicted values, daily administration of dornase alpha has been shown to reduce the risk of respiratory tract infections.29 Dose of dornase alpha is 2.5 mg inhaled via nebulizer once daily. Adverse effects include voice alteration and rash.29
Hypertonic saline is a sterile sodium chloride (NaCl) solution available as 3%, 3.5%, and 7% concentrations, administered via a nebulizer.30,31 Administration of hypertonic saline creates an osmotic gradient within the airway, drawing water into the airway surface liquid and partially rehydrating it.32 This increases the volume and liquidity of airway secretions, improving ciliary function and promoting mucus clearance.32 Research shows inhalation of 2-4 mL of hypertonic saline twice daily results in fewer pulmonary infections.32 It is recommended for patients 6 years or older.33 However, a recent Cochrane Review found low to very low‐certainty evidence showing lung improvement and function, at least in the short term. Cochrane Review also suggests it should not be used in preference to dornase alfa.31 The benefit of hypertonic saline appears to be a modest reduction in frequency of pulmonary exacerbations and an improvement in chest symptoms. However, there is no evidence to say for whom it works best. In children under 12 years of age, sufficient evidence was not found to justify its routine use.31 More research is needed. Hypertonic saline may cause cough or throat irritation.33
Mannitol is a naturally occurring sugar alcohol that may be used to improve pulmonary function in some patients.29,34 When inhaled as a dry powder, mannitol draws water into the airway lumen, hydrating the airway surface liquid and loosening secretions.27,28 However, mannitol carries the risk of airway hyperresponsiveness, necessitating a tolerance test before use.35 Mannitol is provided as a dry powder for inhalation, making it a convenient and portable option.29,34
Chronic Airway Infections
Chronic airway infection management in CF patients centers on suppressing persistent pathogens, reducing lung inflammation, and preserving function. The most common pathogens include Pseudomonas aeruginosa, Burkholderia cepacia complex, Staphylococcus aureus, and nontuberculous mycobacteria.13,36 The altered mucus in CF airways harbors biofilm-forming bacteria that are difficult to eradicate.
Treatment is focused on multimodal control rather than cure. Inhaled antibiotics, such as tobramycin solution or powder, are mainstays for patients chronically infected with Pseudomonas aeruginosa. These are often administered in alternating cycles (e.g., 28 days on and 28 days off) to suppress pathogen burden and reduce the frequency of acute exacerbations.12 Intravenous or oral antibiotics are reserved for acute exacerbations or pathogens less amenable to inhaled therapy, with antimicrobial selection guided by culture sensitivities and individual patient history.12
Table 3 lists examples of common pathogens and antibiotics used in treatment.37 This is not a comprehensive list. For comprehensive safety and efficacy information, consult drug prescribing information. Culture and susceptibility testing are recommended to tailor treatment.
Adjunctive therapies include mucolytics (e.g., dornase alfa and hypertonic saline) and ACTs to reduce mucus viscosity and enhance airway clearance.25 CFTR modulators have revolutionized therapy for eligible patients, restoring some ion channel function and potentially reducing the risk of infection.25 Routine surveillance of airway microbiology is emphasized for guiding therapy and rigorous infection control strategies—both in clinical settings and the community.39 Guidelines recommend microbiologic surveillance for bacteria every three months and mycobacteria annually. For allergic bronchopulmonary aspergillosis (ABPA), annual laboratory evaluations are recommended.39 This approach is crucial for minimizing the spread of resistant organisms.39
Table 3
Common Bacteria and Select Antibiotics Used to Treat Airway Infections in CF Patients27,37,38
| Pathogen | Antibiotic |
| Staphylococcus aureus | Amoxicillin and clavulanic acid (PO) Cephalexin, cefdinir (PO) Meropenem (IV) Tetracycline (PO) |
| Pseudomonas aeruginosa | Piperacillin tazobactam (IV) Tobramycin (inhaled) Azithromycin (PO, IV) Ciprofloxacin (PO, IV) |
| Methicillin-Resistant Staphylococcus aureus | Sulfamethoxazole/ trimethoprim (PO) Vancomycin (IV) Linezolid (PO, IV) Doxycycline (PO) |
| Nontuberculous mycobacteria | Azithromycin (PO, IV) Amikacin (inhaled, IV) Ethambutol (PO) |
Antibiotic pharmacokinetics (PK) in CF patients differ from those of individuals without CF, mainly in distribution and clearance. Oral absorption of most antibiotics does not change significantly. CF patients exhibit an increased volume of distribution for many drugs, due to increased lean body mass and altered tissue binding.24 Antibiotics are cleared more rapidly due to increased renal clearance. This results in shorter drug elimination half-lives and lower serum concentrations for standard doses, necessitating higher or more frequent dosing regimens to achieve therapeutic levels.24
Antibiotic treatment requires individualized dosing and careful therapeutic drug monitoring to balance efficacy with risk of toxicity. PK variability in CF is high, and dosing considerations should account for differences in age, nutritional status, and organ function.24 Understanding and adapting to the altered PK profile is fundamental to effective CF infection management.
Chronic Airway Inflammation
Anti-inflammatory agents are used in managing airway inflammation associated with CF.40-42 High-dose oral ibuprofen has demonstrated efficacy in slowing pulmonary function decline among pediatric CF patients, primarily by inhibiting neutrophil-driven inflammation.41,42
The daily maximum dosage for treating inflammation, according to current prescribing information, is 3,200 mg.40,42 Some clinicians suggest a lower daily maximum dose.41 Careful monitoring is necessary when using high-dose ibuprofen due to the potential adverse effects.40-42 The adverse effects also reduce patient adherence.42 Therefore, when treating patients with 3,200 mg/day, the clinician or health care team should assess the patient’s adherence to the treatment plan and observe sufficient clinical benefits to offset the potential increased risks.42
Azithromycin is routinely prescribed for its immunomodulatory effects, which help enhance lung function and reduce the frequency of pulmonary exacerbations in individuals with CF.43 Its mechanism involves reducing pro-inflammatory cytokines and modulating neutrophil activity, with pronounced benefits observed in patients experiencing chronic Pseudomonas aeruginosa infection. Azithromycin improves lung function, reduces pulmonary exacerbations (PEx), and improves quality of life.43
Systemic corticosteroids, most often administered orally, may be used to attenuate inflammation during acute exacerbations; however, prolonged use is generally avoided because of significant long-term risks, including growth suppression, impaired glucose tolerance, and osteoporosis.36 While inhaled corticosteroids are commonly used, the supporting evidence for their routine effectiveness in CF remains limited.36 Their administration is typically reserved for cases involving coexisting asthma or reactive airway indications.
Table 4
Anti-Inflammatory Treatment for Cystic Fibrosis12,40,44-46
| Medication | Dose | Common Side Effects |
| Aztreonam | 75 mg nebulized 3x/day for 28 days if age 7+ | Cough, nasal congestion, wheezing, pharyngolaryngeal pain, chest discomfort |
| Tobramycin | 1 ampule (300 mg) inhaled 2x/day for 28 days | Nephrotoxicity, ototoxicity, cough, pharyngitis, dyspneaᵞ, hemoptysis, asthma, rales |
| Azithromycin† | 250 mg (if < 36 kg) or 500 mg (if > 36 kg) 3x/week | Diarrhea, nausea, vomiting |
| Ibuprofen† | 20-30 mg/kg 2x/day Max 3,200 mg/day | Abdominal pain, constipation, edema, neutropenia, prolonged bleeding time |
†Off-label use for CF
Antibiotic therapy and mode of delivery (enteral, inhaled, and/or intravenous) are dictated by the severity of the exacerbation and previous/current respiratory culture results.3,38 In patients (aged ≥ 6 years) with chronic presence of Pseudomonas aeruginosa (P aeruginosa) in airway cultures, prophylactic use of antibiotics (tobramycin, aztreonam, and azithromycin) is recommended.47 The use of inhaled tobramycin and aztreonam delivers the drug locally to the lung and decreases the risk of systemic side effects.47
For chronic use, tobramycin is administered by nebulization (300 mg) or dry powder inhalation (112 mg) twice daily for 28 days on and 28 days off.38 It can also be administered IV or IM at a dose of 10 mg/kg/day given in four equally divided doses.38
Aztreonam inhalation solution is an alternative antibiotic for patients with chronic colonization of P aeruginosa.44 Aztreonam 75 mg is administered by nebulization route three times daily for 28 days on and then 28 days off.44 If a patient is chronically using a bronchodilator and a mucolytic, the bronchodilator should be used first, followed by the mucolytic, and then the aztreonam.44,48
Pulmonary CF Complications
Pulmonary Exacerbation
Pulmonary exacerbations are a common complication of CF lung disease but lack a universal definition, making treatment standardization challenging.49 Pulmonary exacerbations present with increased respiratory symptoms—such as coughing, sputum production, and wheezing—and reduced lung function and fatigue.3 Symptoms are linked to lower quality of life, more hospitalizations, and decreased survival.49
Treatment includes antibiotics, sometimes in combination to achieve synergistic activity and reduce drug resistance.50 Alternative treatments, such as the cephalosporin/b-lactamase inhibitor combinations ceftazidime-avibactam and ceftolozane-tazobactam or others, may be required.50 Increased frequency of ACT helps clear secretions from the airways.3
Hemoptysis
Coughing up blood or bloody mucus (hemoptysis) results from chronic infection and inflammation, which cause the erosion of hypertrophied bronchial arteries into the airways.3 Vitamin K deficiency can contribute to hemoptysis.3
Mild to moderate hemoptysis is defined as expectoration of sputum volume ≤ 240 mL. Management includes antibiotics and consideration of limiting certain exacerbating treatments, such as ibuprofen, hypertonic saline, dornase alpha, and ACT.3,49 Severe hemoptysis (> 240 mL) is considered life-threatening and causes significant distress.3,49
When symptoms are severe, patients are admitted to the hospital for treatment, which typically includes intravenous antibiotics and discontinuation of airway clearance and aerosolized therapies.49 Procoagulant options, such as tranexamic acid and aminocaproic acid, have been associated with decreased hospitalization rates, shorter hospital stays, and reduced need for invasive procedures.49
Pneumothorax
Pneumothorax is a serious and common complication in CF patients, particularly those with infection or massive hemoptysis. It arises from air trapping, where high alveolar pressure causes air to leak into the pleural space, resulting in acute chest pain and dyspnea.3 Symptoms may be severe or mild. Early and decisive medical or surgical management is advised after the first episode if the patient is suitable for intervention.3
Therapeutic options include intercostal tube drainage and video-assisted thoracoscopic surgery (VATS).51 Chronic nebulized mucolytics and antimicrobials should be continued.49 No consensus exists regarding the administration of oral or IV antibiotics.49 Cystic fibrosis patients with pneumothorax should not fly on a plane, perform spirometry, or lift weights for the 2-week period after the pneumothorax has resolved.49
Chronic Rhinosinusitis (CRS) and Nasal Polyposis
Sinus disease is common in patients with CF due to defective CFTR protein, whereas nasal polyposis varies and tends to increase with age.3 Rhinosinusitis and polyposis may be intermittent or chronic, often caused by mucus blockage and presenting as headache, congestion, or facial pressure.3,52
Treatment involves saline nasal irrigation and nasal steroids to clear mucus and reduce inflammation.3,52 Surgery may help severe cases. Ciprofloxacin and azithromycin are standard antibiotics for prevention and control, and leukotriene receptor antagonists can be used with topical steroids as alternatives to oral corticosteroids for chronic rhinosinusitis with polyposis.52
Transitioning to Adulthood
Understanding self-care skills and transitioning to adult care should begin in the early teenage years.3 Readiness assessment tools and questionnaires are available for self-management skills. One is the Transition Readiness Assessment Questionnaire (TRAQ).3,53 Discussions should also include educational/vocational plans, behavioral risk counseling, screening for depression/anxiety, and reproductive health and family planning.3,54
Adolescents should be provided a meaningful opportunity to participate in the design and delivery of treatment plans.55 Meeting the adult CF team before transition can decrease anxiety and concern.55 In preparing for the transition, the patient's knowledge of CF should be assessed so the patient may be provided with age-appropriate educational materials.55
Patient Counseling
Helping patients and caregivers understand and implement CF treatment strategies ensures more effective management of CF and improves overall outcomes and quality of life. Table 5 lists important counseling points.
Table 5
Cystic Fibrosis Patient Counseling Points16
| Category | Counseling Points |
| Disease Education | Discuss the genetic nature of CF and its multi-organ effects, emphasizing why routine management and lifelong care are necessary. Encourage patients and caregivers to learn about CF and seek reliable resources and support continuously. |
| Lung Health and Airway Clearance | Demonstrate airway clearance techniques: These might involve chest physiotherapy, mechanical devices like vibrating vests, and special breathing exercises. Stress the importance of performing these routines regularly (often multiple times daily) to loosen and expel thick mucus, minimize lung infections, and improve airway function. |
| Nutrition and Exercise | Counsel on the need for a high-calorie, high-fat diet to combat malabsorption and maintain weight. Regular aerobic exercise Hydration helps thin lung mucus and is also needed for extra salt/fiber during hot weather or exercise. |
| Medication Management | Stress adherence to prescribed antibiotics, mucus-thinning medications, inhaled therapies, and digestive enzymes. Missing doses can lead to complications. Suggest organizational tactics like medication schedules, apps, or pill boxes. Encourage reporting side effects for therapy adjustments. Instruct patients on the proper techniques for storing and handling inhaled, oral, and IV treatments, and on protecting medications from light and heat, if appropriate. Attention should be paid to refrigeration requirements, and ampules should be checked for leaks before use. |
| Infection Risk and Prevention | Emphasize ways to reduce infection risk: frequent handwashing, avoiding contact with sick individuals, and keeping up-to-date with all recommended vaccines. Advise the patient to avoid exposure to smoke and other lung irritants. |
| Routine Follow-Up | Emphasize the need for regular clinic visits (often every 3 months) for comprehensive checkups, lung monitoring, and therapy adjustment. Encourage patients to keep records of symptoms, medications, and medical history for more effective and personalized care. |
If taking tobramycin, advise patients to inform their physician if they experience ringing in the ears, dizziness, or any changes in hearing.45 Advise patients to inform their physician if they have any history of kidney problems because tobramycin inhalation solution is in a class of drugs that have caused kidney damage.45
Counseling and patient aids, such as auxiliary labels, assist with patient education.56,57 Auxiliary labels on ibuprofen should indicate that it should be taken with food or milk.58 Review the patient record for any possible interactions, and ensure the patient and family are not purchasing an over-the-counter (OTC) medication containing another nonsteroidal anti-inflammatory (NSAID) or aspirin at the same time. Watch for other OTCs that may increase the risk of GI bleeding. A nurse or pharmacy technician should refer the patient, who is considering using an OTC drug, to the pharmacist or physician for an assessment of possible drug interactions.
Patients should be asked to describe the order in which they administer inhaled medications.59,60 Bronchodilators, for example, are generally administered before inhalation of hypertonic saline or mannitol. Dornase alfa is generally used before airway clearance procedures.59,60
Hypertonic saline can damage electrical equipment, such as computers. Advise patients to take hypertonic saline in a well-ventilated area, away from electronic devices.59 Women with advanced CF contemplating pregnancy should carefully consider the risks in consultation with high-risk obstetrics and CF providers.
There are many support opportunities for families, patients, and clinicians. Table 6 provides select support resources to review and consider sharing.
Table 6
Support Resources
| Organization | Resource |
| Cystic Fibrosis Foundation | https://www.cff.org/ and in the mobile App Store |
| Cystic Fibrosis Engagement Network | https://www.engagecf.org/ |
| The Rock CF Foundation | https://letsrockcf.org/ |
| CF@Home (self-management) | In the mobile App Store |
| CF Rise (multi-language tools, for ages 10-25), for teen→adult transition | https://www.cfrise.com/ |
Next Steps
Pulmonary disease continues to account for almost 60% of CF-related mortality.3 Lung transplantation can extend and improve the quality of life of individuals with CF. Recent reports reveal that individuals with CF are experiencing an additional 9.5 years of median survival following lung transplantation.3
Cystic fibrosis research is ongoing. In one study, researchers are striving to identify children at risk of developing CF and to assess children who carry a mutation at risk of CFTR-related disorder (CFTR-RD) but remain asymptomatic during childhood.61 Another study is investigating whether the relatively low number of reported COVID-19 cases among people with CF (pwCF) is due to enhanced infection-prevention practices or to protective genetic or immune factors in pwCF.62
Summary
Cystic fibrosis is a rare genetic disorder caused by mutations in the CFTR gene. It primarily affects the lungs and digestive system. Thick mucus production leads to chronic respiratory problems and frequent infections, with symptoms such as a persistent cough, recurrent lung infections, and malabsorption. Early diagnosis through newborn screening, genetic testing, and regular lung function monitoring is crucial for timely intervention and improved outcomes.
Advances in CF care have significantly increased life expectancy, now ranging from 50 to 65 years for most patients in the United States. Treatment strategies focus on multidisciplinary management, including airway clearance, infection control, anti-inflammatory therapy, and nutritional support. CFTR modulator therapies, tailored to specific genetic mutations, have revolutionized care by enhancing lung function and reducing complications.
Effective management of CF involves a collaborative healthcare team and patient education on treatment adherence, airway clearance, infection prevention, and nutrition. Pharmacists and technicians are essential in medication management and patient support. Comprehensive resources and counseling help individuals and families navigate care and transition into adulthood, promoting long-term health and quality of life.
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