Inhaled Therapy Choices Expand Treatment Opportunities in Cystic Fibrosis

A recent review entitled “Inhaled therapy in cystic fibrosis: agents, devices and regimens” published in the journal Breathe explored the increasing number of inhaled therapeutic options for patients with CF, and how innovations in these therapies are dramatically improving quality of life for those with the disease.

Historically, patients with cystic fibrosis had to engage in lengthy physiotherapy procedures and suffered from chronic infections with opportunistic infections, such as Pseudomonas aeruginosa. They also underwent nebulizer treatments that took a great deal of time to use, required extensive cleaning between treatments, and were often not adhered to according to the doctor’s recommendation.

In recent years, newer methods of delivering medications to cystic fibrosis patients have come into use.  These changes in drug delivery lead to faster-acting therapies, are successful in managing patients with CF, and perhaps more importantly, they result in greater compliance with cystic fibrosis treatments.

The two main delivery systems that are an increasing part of CF therapy are “smart nebulizers” and “dry powder inhalers.”  These systems have increased the amount and variety of drugs given to CF patients and involve faster delivery systems that take less time to deliver than previous modes of therapy, increasing patients’ compliance with therapy and improving quality of life.

In “smart nebulizer” therapy, the nebulizer delivers more precise amounts of the drug over a shorter period of time than regular nebulizers.  Drugs can be delivered only during inhalation and not during exhalation, which simply wastes drug.  Patients are taught how to use these nebulizer devices so that exhalation is through the nose and precise particle sizes are instilled into the patient’s bronchial tree, getting to the lower airways fairly effectively.

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Conversely, in “dry powder inhalers,” medications can be quickly delivered and are portable.  Dry powder inhalation devices can be created to deliver medications that thin the mucus of the airways and antibiotics, which have been shown to decrease colonization of pathogenic bacteria in CF patients.  There are many different antibiotics that can be delivered through dry powder inhalation that reduce infections with opportunistic pathogens like Pseudomonas aeruginosa.

Using both of these new therapies, CF patients can quickly receive their daily treatments, which are custom tailored to the patient’s specific needs.  Compliance has been shown to increase dramatically with these forms of therapy when compared to older delivery systems and physiotherapy.

Ideally, CF medications are given in a specific order:

• Bronchodilator therapy, which opens the airways for the passage of other medications.
• Mannitol and hypertonic therapy treatments, which help clear the airways and loosen airway mucus.
• Inhaled antibiotics, which reach the lower airways better than if the airways haven’t been cleared out.
• rhDNAse, which can be given prior to or after airway clearance to improve CF symptoms.

It is believed that these types of therapies will shorten treatment time and improve medication compliance in CF patients, who will experience an improved quality of life, decreased morbidity and decreased mortality.