Cystic Fibrosis and Sinusitis
Jane Wang, RN, MSN, FNP
Stanford Otolaryngology Department
Stanford Sinus Center
Sinus disease is common among patients with CF. With improved understanding of CF and sinusitis, medical and surgical interventions should be carefully selected to relieve sinus symptoms and to improve a patient’s quality of life.
What is sinusitis?
The sinuses are four paired, air-filled cavities located between the eyes and below the brain called the maxillary, ethmoid, sphenoid and frontal sinuses. The sinuses drain through small openings called ostia that are typically only 2-4mm in diameter. The sinuses have multiple functions, including to: warm and humidify the air that we breathe; trap and filter particles in the air; reduce the heaviness of the skull; and provide resonant chambers for our voice. Sinusitis is a disease characterized by inflammation and infection of the mucous membranes and underlying bone of the nasal passages and sinus cavities .
Why do CF patients have sinusitis?
The nose and sinuses constantly produce mucus that are swept by the cilia and drain through the sinus ostia. The constant, rhythmic movement of the cilia keeps the sinuses clean and healthy. When this does not occur, mucus accumulates, leading to inflammation and infection. CF patients produce very thick, tenacious mucus, which often results in impaired mucus clearance, mucus stagnation, bacterial colonization, chronic inflammation, mucosal swelling, and ostia obstruction.
How is sinusitis diagnosed?
Sinusitis can be diagnosed in several ways:
Symptoms of sinusitis may include facial pain or pressure, nasal obstruction, discolored and thick nasal discharge, poor sense of smell, headache, bad breath, fatigue, cough and dental pain to the upper teeth. Just like CF itself, sinusitis among CF patients also presents with wide variation in its severity, from being asymptomatic to debilitating.
Sinus specialists use a nasal endoscope to examine the nasal and sinus cavity. Nasal polyps, which are fluid or mucus filled benign masses, are the most distinctive physical finding, occurring in up to one-third of CF patients . Depending on their size and location, polyps can be very problematic. They frequently cause obstruction of the sinus drainage passage, contributing to abnormal mucus clearance among CF patients. Thick, discolored nasal discharge is frequently seen upon nasal endoscopy as well.
Sinus computed tomography (CT) scan
More than 90% of patients with CF exhibit radiographic evidence of chronic sinusitis showing the sinus cavity filled with mucus or polyps (rather than air). Chronic sinusitis often causes underdevelopment of certain sinuses among CF patients . Despite of the abnormal sinus CT scans in most CF patients, only about 10-20% of patients are symptomatic from their sinus disease .
Sinus culture obtained during nasal endoscopy is helpful to diagnose sinus infection. It also serves to determine culture-directed antibiotic treatment, especially in suspicion of resistant organism. Common organisms in CF sinusitis include Staphylococcus aureus, Pseudomonas aeruginosa, Haemophilus influenza, Burkholderia cepacia, Achromobacter xylosoxidans, and Strenotrophomonas maltophilia.
What are the medical treatments for sinusitis?
Treatment of CF related sinusitis takes on many forms, including antibiotics and anti-inflammatory agents. Both can be taken either orally or delivered topically via nasal spray, nasal irrigation and a nebulizer. Obviously, topical agents tend to be more effective in patients whose sinuses are unobstructed; these patients typically have had sinus surgery. Topical agents may potentially avoid systemic side effects associated with oral medications, and can potentially treat resistant bacteria without intravenous antibiotic use. Allergy therapy may also be considered simultaneously when appropriate. For treatment to be effective, patients need to develop and stick to a consistent regimen.
When to consider surgery for CF patients with sinusitis?
At the Stanford Sinus Center, maximal medical therapy for sinusitis includes three to six weeks of broad-spectrum culture-directed antibiotics, topical nasal steroid, saline nasal irrigation, allergy therapy if indicated, and two weeks of concurrent oral steroid use in some patients. Sinus surgery is considered when a patient exhibits the following: persistent sinus and pulmonary symptoms despite maximal medical therapy; evidence of sinus disease on post-maximal medical therapy sinus CT; endoscopic evidence of persistent sinus disease, such as anatomical abnormalities, pus, extensive polyps, etc. Goals of endoscopic sinus surgery are to enlarge sinus drainage pathways; remove polyps; break the cycle of obstruction leading to chronic sinusitis; and improve lung function [10, 11]. It’s important to understand that sinus surgery is an adjunct, but not a substitute for medical therapy.