Cystic Fibrosis (CF)

Cystic fibrosis (CF) is an inherited, life-limiting condition that affects several body systems. Respiratory dysfunction is the most common manifestation of CF and the main cause of death. The aberrant output of mucus in the airways results in airway blockage and mucus plugging, which can lead to bacterial infection and the further production of mucus. Over time, this process causes significant airway damage and destroys pulmonary function. Airway clearance, or the removal of secretions from the airways, is thus an important part of the treatment of CF.1 

Many advances have been made in the treatment of CF. Medications that thin and clear heavy mucus from the airways are available, as well as enzymes that aid fat and mineral absorption and antibiotics that treat infections. In addition, some novel treatments focus on the cystic fibrosis transmembrane conductance regulator (CFTR) protein.2 Airway clearance can be accomplished in a variety of ways, such as with mechanical devices and physical manipulation during physiotherapy.1


Bronchodilators are prescribed to more than 80% of patients with CF, and many find them helpful in relieving wheezing and dyspnea.3 These drugs are administered with either a metered-dose or dry powder inhaler or with a nebulizer.4 

Inhaled bronchodilators, both short- and long-acting, are used to manage respiratory symptoms, open the airways, and facilitate airway clearance. They may be prescribed along with physiotherapy to ease the symptoms of CF asthma, facilitate sputum clearance, and minimize symptoms of bronchoconstriction during nebulizer therapy. Short- and long-acting inhaled bronchodilators differ in their duration of action. Short-acting bronchodilators are used to treat abrupt attacks of breathlessness. They work quickly, and the effects last for 4 to 6 hours with beta-2 agonists and 6 to 8 hours with muscarinic antagonists. For longer-lasting effects and maintenance, long-acting bronchodilators are used as prophylaxis.3

cystic fibrosis inhaler
An inhaler for the treatment of cystic fibrosis. Credit: Getty Images


Bronchodilators are administered with a metered-dose inhaler. This device releases a specific dose of medication into the lungs. A nebulizer or a dry powder inhaler may be used less often to administer medication.4 Bronchodilators are frequently packaged in an aerosol canister. A spacer, which is a tube attached to the canister, may be used along with the inhaler to ensure that most of the drug reaches the lungs rather than remain in the posterior portion of the mouth or the throat. To avoid the transmission of pathogens, all equipment must be disinfected after it is used to administer a bronchodilator. Most patients inhale 2 puffs of their bronchodilator at intervals of 1 minute.4-6 

View of portable nebulizer for the treatment of cystic fibrosis. Credit: Getty Images


Nebulizers convert liquid medicine into a fine mist, which is then inhaled. In many cases, the instructions that accompany an inhaled drug advise that the drug be administered only with a specific nebulizer. Patients should always use a nebulizer that has been specified by the manufacturer of their medication.7 Ultrasonic nebulizers, jet nebulizers, breath assist open vent jet nebulizers, breath-actuated jet nebulizers, and vibrating mesh nebulizers (Aerogen Aeroneb® [OnQ technologyTM] and Pari eFlow® [touch spray technologyTM]) are some products currently on the market.8 

To prevent an infection from reaching the lungs through a nebulizer, it is essential that the nebulizer be kept clean and disinfected. Patients with CF must have their own nebulizers, and respiratory treatments must be administered in a separate room to avoid spreading germs to others.7 

The age, lifestyle, level of compliance, desire for treatment, and cognitive and physical capacity of a patient, as well as the patient’s caregivers, must be considered when an appropriate nebulizer is selected. The patient’s breathing pattern, in addition to the properties of the nebulized medication, nebulizer output, and cost and lifespan of the nebulizer device, should also be taken into account.8

Inhalation Accessory Devices

The 2 types of inhalation accessory devices (IADs) are spacers and holding chambers. A spacer is an add-on component that allows the aerosol plume of a metered-dose inhaler to widen and decelerate, so that it is transformed from a high-pressure actuation spray into a fine mist. The mist remains in the spacer until the patient breathes it in through a one-way valve, bringing the medication into the lungs.9 

IADs offer a number of benefits. They improve drug delivery, compensate for poor technique, and reduce oropharyngeal deposition. However, they also have various drawbacks. Because of the large size and volume of an IAD, bacterial contamination is a possibility. The device must be cleaned regularly, and electrostatic charges may limit the delivery of medicine to the lungs.9 

Vascular Access Devices

Airway Clearance Device for CF
A young man at home uses a machine to help with mucus buildup from cystic fibrosis. Credit: Getty Images.

Patients with CF who require intravenous antibiotic treatment for more than a few days, such as during an exacerbation, may benefit from vascular access devices. Regular intravenous lines become contaminated, strain small veins, or fall out of a vein after a few days. Vascular access devices such as peripherally inserted central catheters (PICCs) can be used for days, weeks, or even months. Implantable devices, like ports, are designed to last a long time. They are made of a material that does not easily become contaminated and will stay in place if properly cared for. Furthermore, the rate of complications is low.10

Airway Clearance Devices

Numerous devices are available to help patients with CF clear their airways. Unlike manually administered chest physical therapy, these devices can be used by patients without the assistance of a healthcare practitioner or caregiver.11 Positive expiratory pressure (PEP) devices, airway oscillating devices (AODs, handheld or stationary), and high-frequency chest compression (HFCC)/mechanical percussion (MP) devices are the 3 types of airway clearance devices.1 


  1. Airway clearance devices for cystic fibrosis. Ont Health Technol Assess Ser. 2009;9(26):1-50.
  2. Treating and managing cystic fibrosis. American Lung Association. Updated March 12, 2020. Accessed January 22, 2022. 
  3. Smith S, Edwards CT. Long-acting inhaled bronchodilators for cystic fibrosis. Cochrane Database Syst Rev. 2017 Dec;2017(12):CD012102. doi:10.1002/14651858.CD012102.pub2
  4. ALBUTEROL SULFATE HFA- albuterol sulfate aerosol, metered. DailyMed. Updated April 14, 2021. Accessed January 20, 2022.
  5. Bronchodilators. Cystic Fibrosis Foundation. Accessed January 22, 2022.
  6. Types of bronchodilators for cystic fibrosis treatment. Reviewed November 2021. Accessed January 22, 2022.
  7. Nebulizer care at home. Cystic Fibrosis Foundation. Accessed January 22, 2022.
  8. Collins N. Nebulizer therapy in cystic fibrosis: an overview. J R Soc Med. 2009;102(Suppl 1):11-17. doi:10.1258/jrsm.2009.s19003
  9. Buddiga, P, Kaliner MA. Use of metered dose inhalers, spacers, and nebulizers. Practice essentials, Medscape. Updated April 12, 2020. Accessed January 22, 2022.
  10. Vascular access devices: PICCs and ports. Cystic Fibrosis Foundation. Accessed January 22, 2022.
  11. Marks L, Laube J. Cystic fibrosis treatment. Everyday Health. Reviewed April 2, 2018. Accessed January 22, 2022. 

Reviewed by Debjyoti Talukdar, MD, on 1/26/2022.