Cystic Fibrosis Center NEWS SUMMER 2003 Sinusitis and Cystic Fibrosis Introduction Respiratory disease in cystic fibrosis (CF) is usually associated with the lungs. However, acute and chronic sinusitis are common complications that can cause significant symptoms that sometimes adversely impact the lungs. Sinusitis is defined as inflammation of the nose and sinuses. Most persons with CF develop sinus symptoms, with onset typically between the ages of 5 and 14. Severity of symptoms and disease varies widely. Treatments include oral, systemic and inhaled medications as well as surgical and medical interventions. In this issue, we will discuss CF-related sinus symptoms, diagnoses and some treatment options that may minimize the discomfort caused by sinusitis. The lungs, nose and sinuses comprise the respiratory tract, and all can be impacted by CF genetic mutations. Anatomically, the lungs comprise the “lower respiratory tract” while the nose and sinuses comprise the “upper respiratory tract.” By evaluating the respiratory tract as one continuous structure, it is easier to understand the causes and treatment of sinus symptoms and disease in persons with CF. Continued on Next Page INSIDE FREQUENTLY ASKED QUESTIONS PAGE 5 LPCH EDUCATION DAY HIGHLIGHTS PAGE 6 PHASE IIB GENE THERAPY TO BEGIN IN JUNE PAGE 7 Devon Wakefield has CF sinusitis and is a dedicated and enthusiastic soccer player. He receives monthly sinus flushes at LPCH. Sinusitis and Cystic Fibrosis The Sinuses in Cystic Fibrosis The sinuses consist of 4 pairs of air filled spaces: the ethmoid, Ethmoid sinuses maxillary, sphenoid and frontal sinuses, located behind the nose, cheeks and eyes. The sinuses warm and humidify the air you Sphenoid sinus breathe. They also filter particles in the air, such as bacteria, Frontal sinus spores and dust. The sinuses, like the lungs, are lined with epithelia cells that contain the defective CFTR that contributes to the inflammation and infection that causes CF complications. Most people with CF suffer from pansinusitis, inflammation and infection of all the sinuses. Maxillary sinus The incidence of sinusitis in CF is not known, but the great majority develop sinus symptoms. Symptoms include: The incidence of “hay fever” or allergic rhinitis is the same I nasal obstruction as the general population. There is no known association of I chronic congestion or discharge allergies causing nasal polyps or the development of asthma or I headaches more serious lung disease. However, since about 20% of persons with CF also have allergies, it is important to pay I post-nasal drip with morning cough attention to known allergies or seasonal worsening of sinus I cough that is aggravated by lying down symptoms so that they can be treated effectively. I severe bad breath Diagnosis I constant need to “clear one’s throat” Diagnosis of CF-related sinusitis is based on symptoms. Adolescents and adults are more likely than children to Imaging studies may help determine the extent of disease. report sinus-related headaches that often don’t respond to Sinus endoscopy, placement of a small camera into the typical headache remedies. Acute sinusitis is the term used maxillary and sometimes ethmoid sinuses for direct viewing, when symptoms become severe. Many persons with CF have is often used by an otolaryngologist (ear, nose and throat chronic, or recurring, sinusitis. specialist) to visualize sinus polyps and obstructions. Nasal and sinus polyps, benign masses of tissue, occur in as Treatment many as 48% of persons with CF and increase in frequency Treatment includes antibiotics, nasal steroids, clearance with age. Polyps can grow over time, and often obstruct with saline or antibiotic flushes, antihistamines and drainage of the nose and sinuses. decongestants and surgical interventions. Although there is no ‘best’ treatment for CF sinus disease, most people will CF sinusitis is distinctive in imaging studies. Improper benefit from a combination of therapies. Modalities are the formation of the frontal sinuses, possibly a result of sinus same as those used for non-CF patients, although dosing and obstruction early in life, is common and can be seen on x-rays. choice of drugs may be different, depending on the specific In CT and MRI scans, more than 75% opacification pathogens found in a culture. (cloudiness) of the maxillary and ethmoid sinuses is a hallmark of CF. Abnormal paranasal sinuses also are common. Like the lungs, the sinuses benefit from “airway clearance.” This includes anything to help remove mucus, such as nose CF-related sinus infections generally are distinct from blowing, saline flushes, saline nasal sprays to moisturize non-CF bacterial sinus infections. Just as in CF lung infections, mucus so it can be “blown out” more easily and the most common sinusitis bacteria are Staphylococcus aureus; ENT-assisted interventions that remove mucus. More Haemophilus influenza; Pseudomonas aeruginosa; Burkholderia aggressive interventions are often recommended for people cepacia; Achromobacter xylosoxidans and Stenotrophomonas with frequent pulmonary exacerbations. maltophilia. People with CF can have high bacterial loads in their sinuses without experiencing symptoms. Non-bacterial Antibiotic treatment for CF sinusitis is similar to that for infectious agents are also found in CF sinusitis. Aspergillus lung exacerbations. Choice of antibiotic is based on the molds colonize up to 40% of sinuses in adults with CF, although specific pathogens and their drug resistances. You should they often do not cause symptoms. Persons with CF are not consult your CF physician before an antibiotic course is more susceptible to common viral upper respiratory infections initiated for sinusitis to ensure the drugs, duration and dosing (the “common cold”). are appropriate. Combination antibiotic therapies are often 2 Lucile Packard Children’s Hospital recommended and dosing can be significantly different than treatment of non-CF sinusitis. Nasal swabs and transantral aspirations for cultures are used, as well as sputum samples, since typically sinuses are infected with the same organisms as the lungs. Laboratories not associated with a CF Center often are not equipped to identify many CF pathogens and drug sensitivity levels that are important to optimum treatment. Treatment courses are usually 3–6 weeks due to the poor penetration of drugs into the sinuses, but there is no ideal length of therapy. Antibiotics can be delivered systemically, via mouth or vein, or locally by inhalation or direct injection into the maxillary sinuses, a process referred to as antibiotic flushing. Systemic antibiotics are the standard of care, and sinus and pulmonary exacerbations are usually treated simultaneously. A recent study of non-CF patients using antibiotics nebulized directly into the sinuses found decreased nasal bacterial counts and inflammation. For users of inhaled TOBI™, sinus penetration of the drug occurs. Nebulizers adapted for inhalation through the nose coupled with an array of antibiotics are now available, and are reported to help some people with CF. Flushing of the maxillary sinuses through catheters placed directly into the sinus is possible in people who undergo surgical antrostomies to enlarge the sinus openings. In two studies involving lung transplant patients, flushing with tobramycin through antrostomies resulted in absence or delay of contamination of the newly transplanted lungs with Pseudomonas. Another study of CF patients with chronic sinusitis showed that those who had monthly flushes with tobramycin had fewer surgeries over a two-year period of time. What remains unclear is how this treatment compares to other forms of “airway clearance” for the upper respiratory tract and the influence of monthly checks on the sinuses. Some patients have monthly appointments in the ENT clinic for flushes, whereas others do the flushes at home. Some patients report relief of sinus symptoms by flushing with saline. Clearing mucus, infectious organisms and hydrating thick secretions may provide symptomatic improvement. Products include saline-filled squeeze atomizers, some of which contain the moisturizing agent glycerol. More economically, patients can flush with a homemade buffered saline solution. (Recipe: Add 1 teaspoon of salt and one pinch of baking soda to a pint of warm water.) The nostrils can be flushed with the saline using a bulb, catheter-tipped syringe or a specially designed nasal irrigation cup available from allergy supply sources. Anna Messner, MD, administers an antibiotic flush procedure on Devon Wakefield. Continued on Next Page www.lpch.org 3 Sinusitis and Cystic Fibrosis Continued from Page 3 Sinus Care at Stanford and LPCH Nasal steroids help some people with CF-related sinusitis. Acute sinusitis in CF patients can be problematic, even after Regular use of nasal steroids may diminish the size and number surgeries, due to chronic infections, sinus pressure, pain of nasal polyps, and their use after polypectomy (removal of and drainage. The Stanford Sinus Center is a unique clinic polyps) may decrease the rate at which polyps re-form. founded by the ENT physicians at Stanford. These doctors use The effectiveness of antihistamines and decongestants in CF several newer tools to help with the diagnosis and treatment is inconclusive. For some, they are too dehydrating. For others, of sinus infections. Sinus Center physicians who routinely especially those with allergies, antihistamines relieve stuffiness treat CF patients include Winston Vaughn, Director