KEYSTONE, COLO. – Despite claims to the contrary, bronchiectasis is alive and unwell.
"One of the things that I hear time and time again is, ‘I just don’t see much bronchiectasis in my practice,’ but I think it’s because we’re not looking," Dr. Gwen A. Huitt remarked at an allergy and pulmonary diseases meeting.
She advised clinicians that if they are prescribing antibiotics for respiratory exacerbations more than twice a year, and possibly even more than once a year, they should consider underlying bronchiectasis as a possible etiology. By the time bronchiectasis is suspected, it is often too late and the patient has developed resistance to an antibiotic, particularly quinolones and macrolides because they are easy to give patients with recurrent respiratory infections.
A noncontrast CT scan – and not a chest x-ray – is the method of choice to diagnosis bronchiectasis, because it allows proper visualization of dilated bronchi and bronchioles, said Dr. Huitt, director of the adult infectious disease unit at National Jewish Health in Denver, which sponsored the meeting. Her service also screens all patients for cystic fibrosis (CF) and alpha-1-antitrypsin deficiency (Alpha-1) both for levels and phenotype because they’ve found that even phenotypic MZ heterozygotes do not clear infection well.
Once bronchiectasis has been determined, it is important to identify the etiology. In one study involving 150 adults with bronchiectasis, however, the cause was idiopathic in 53% (Am. J. Respir. Crit. Care Med. 2000;162:1277-84). That percentage has fallen only slightly since the study was published, she said.
Treatment goals should aim to reduce or eliminate the underlying host deficiency and improve secretion clearance with any and all mechanisms possible. Secretions can be modified with nebulized hypertonic saline starting at 3%, and even nebulized normal saline to get the heavy secretions out, Dr. Huitt said. Acetylcysteine (Mucomyst) and guaifenesin are also helpful, but dornase alfa (Pulmozyme) is not indicated in non-CF patients and was actually harmful in one study.
Clinicians also need to be diligent about controlling infections rationally.
"That means if you have a gram-negative organism, don’t keep throwing cipro [ciprofloxacin] at it because you are going to lose cipro after the fifth or sixth time you give it," she said. "These organisms are going to develop drug resistance.
"This is where using dual therapy with oral antibiotics and inhaled antibiotics, I think, is going to be the cornerstone."
Approval of inhaled ciprofloxacin is right around the corner, and clinical trials of inhaled mannitol and aztreonam are now underway in non-CF bronchiectasis. Inhaled tobramycin (TOBI), amikacin (Amikin), and colistin are established in clinical practice but can be difficult to obtain for non-CF patients, and the brand-name versions can be cost prohibitive out of pocket at about $5,000 a month, Dr. Huitt observed.
"As we expand some off-label uses, and that’s what many of us do when we have very difficult patients to manage and your back is against the wall, you’re going to try some of these things if they are available to you," she said.
Although guidelines do not recommend obtaining sputum cultures in patients with acute exacerbations, she suggests this is for "garden variety" bronchiectasis.
"Again, if someone is coming into your office two times or more a year with a respiratory tract infection, you should really do a sputum culture to see what you’re dealing with," Dr. Huitt insisted.
Clinicians also should think about prevention in the form of immunizations, early treatment of infection and smoking cessation, and get respiratory therapists involved to evaluate patients to see what type of airway hygiene can be utilized.
Although the Flutter valve is a durable and easy-to-use device, National Jewish has drifted away from it because patients with poor lung function cannot lift the ball bearing on it and it can only be used in the upright position, whereas the Acapella valve can be used in multiple positions, Dr. Huitt said.
Inhaled corticosteroids and long-acting bronchodilators can be used for management, but azithromycin (Zithromax) should be used only if there are no nontuberculosis mycobacteria (NTM) on culture.
"Azithromycin is being used like water today and I think it is going to come back and haunt us in the future because we are seeing a huge rise in our service of macrolide-resistant nontuberculosis mycobacteria patients who have been on chronic macrolides as an anti-inflammatory that never had a culture done," Dr. Huitt said. "By the time we see them, they’ve lost the macrolide and that is a cornerstone drug for the treatment of NTMs."