PCD Foundation Blog

Many of you may not know that Dr. Ken Olivier at NIH got involved in the PCD study due to his interest in NTM infections. He was seeing patients with NTM disease with no underlying disorder identified to explain their infections. He hypothesized (having been trained at Chapel Hill) that undiagnosed CF, PCD or Alpha-1 might be involved and worked with UNC to get his site added to the GDMCC study to look for PCD and atypical CF in adult patients with NTM.

I had the chance to speak with Dr. Olivier briefly at dinner tonight and asked him what he was finding at his site. He said that, based on nasal NO levels, they have been “surprised” at the number of people coming in with NTM infections who appear to actually have PCD. The connection has not been as strong with CF or Alpha-1, probably because the diagnosis of those disorders is a little easier and patients have probably had those diagnoses ruled out prior to coming to see him. We didn’t have time for details, but it is a tantalizing tidbit of info. I’m sure it he will get more into detail about this tomorrow…

One of the best things about this meeting has been the really practical information presented. Keeping neb equipment clean was an interesting topic covered by Dr. Gwen Huitt. For inhaled antibiotics (TOBI, colistin, etc.) National Jewish recommends using only a Pari (http://www.pari.com/) neb cup with filter (which is pretty standard, I believe) and the DeVilbiss Pulmo-Aide nebulizer machine, available online at http://www.devilbisshealthcare.com/index.jsp.

Pari neb cups can be boiled for sterilization, but the tubing is another matter. Since people with bronchiectasis are prone to “water” bugs like Pseudomonas and NTMs, it it important that the tubing be kept dry. Dr. Huitt recommends having seven sets of tubing, one for each day of the week. After use, she has her patients jerry-rig a blow drying system for the tubing by taking a standard rubber glove, cutting a slit in one fingertip and inserting one end of the tubing in the slit. A standard hair dryer is then attached to the wrist end of the glove and hot, dry air blown through the glove and into the tubing for one minute. Then leave the tubing in the open air until the next use. Both Dr. Huit and the RT strongly suggested NOT bagging up tubing for storage, but leaving it out in the air.

Some patients have also pulled their neb cup off the tubing at the end of a treatment and allowed the compressor to keep running for several minutes to dry the tubing. This also seems to work, but the hot, dry air of the blow dryer appears to be the most effective at curtailing bug growth.

*Please note–the following is not intended as an endorsement of any particular form of airway clearance, but is provided for informational purposes only.

At the NTM conference, a respiratory therapist (RT) from National Jewish talked about their preferences for airway clearance and highlighted the need for diligent, daily therapy. This talk was given in the context of patients with bronchiectasis and NTM infections, but much of what she presented would apply equally well to PCD patients.

First off, they really like the Vest (any brand) and the blue (for pediatric or low lung volume patients) or the green (adults) original versions of the Acapella. They are not so keen on the Acapella Choice (the cleanable one) at this point because it tends to break easily. Apparently, the manufacturer is working on this and hopefully will be able to come up with a solution to the breaking issue. The treatment regimen they like is Vesting for 10 minutes, followed by five blows (exhaling only) on the Acapella, followed by huff coughing, then repeat x 1 (for a total of 20 minutes Vest time).

The speaker was very clear about the fact that any form of airway clearance is better than nothing and they don’t want to discourage people from doing whatever therapy they find most effective. However, National Jewish does not generally prescribe IPV devices for patients with bronchiectasis because the “jack hammer” effect can lead to hemoptysis in these patients. Also, cough alone is not as effective in bronchiectatic airways because they become “floppy” and can collapse from the pressure of coughing, trapping mucus. PEP devices like the Acapella essentially use positive pressure to stent the airway open, so mixed with with cough or Vest to loosen secretions you may get good results. The speaKer also made a strong point about not confusing lack of productive cough with failure of treatment and suggested that airway clearance is happening with therapy regardless of whether you notice a demonstrable result.

The blue and green Acapella devices cannot be boiled or sanitized, so it is crucial that you only exhale into them and don’t breath in. For those of us (like me) who have difficulty walking and chewing gum at the same time, remembering not to inhale could present a challenge! Acapella is a vibratory PEP (positive expiratory pressure) device. Other devices in this category include the Flutter valve and the Quake. The Flutter is the old standard, but it is positioning-dependent, meaning that it’s effectiveness is strongly impacted by user ability. The Quake is a newer PEP device that allows the user to control the amount of vibration using a hand-turned crank that looks a bit like a fishing reel. The Quake also requires a certain amount of coordination–must exhale and turn the crank simultaneously–so it is not appropriate for everyone.

Postural drainage (tipping upside down) works great for some people and they do it a lot at National Jewish to help people get sputum samples up. However, you need to be sure you don’t have any reflux issues before embarking on a postural drainage regimen and it is always a good idea to do it on an empty stomach to prevent aspiration!

Coming up next…

“What’s Growing in Your Nebulizer?” or “How I Learned to Stop Worrying and Love My Tubing.”

So you may be wondering why non-tuberculous mycobacterial (NTM) infections (MAC, absessum, MAI, etc.) are of interest to people with PCD. NTMs are nasty and destructive bugs in the same family as Tuberculosis (TB) and there is increasing evidence that bronchiectasis-causing disorders contribute to acquisition of these bugs. At Family Day this summer, Dr. Knowles presented a slide indicating that 20% of PCD patients over the age of 29 in a small review had some form of NTM. Since that review, NTM has also been cultured from adolescent PCD patients. The current recommendation is that all PCD adults and adolescents, regardless of whether they are symptomatic or not, be cultured for NTMs annually. This requires a specialized culture called an AFB culture. There are several labs that do quality cultures. Contact the PCD Foundation for more information.

Denver, November 2007

Great information so far. Here are a few surprises:

1.) There does not appear to be a correlation between the severity of bronchiectasis and acquiring an NTM infection. In fact, it appears that, at least in CF and PCD, most people who acquire NTM (non-tuberculous mycobacterial) infections have mild clinical disease. This really surprised me and also adds to the suspicion that susceptibility to these organisms may be more a matter of genetic pre-disposition than of disease progression or exposure. The same is true of ABPA (allergic bronchopulmonary aspergillosis).

2.) The crucial factor in predicting how well you will respond to NTM treatment is whether or not you culture clarithromycin (Biaxin)-resistant organisms.

3.) The importance of not only getting very regular cultures, but cultures that are sent to appropriate labs was reiterated over and over again. This is not just true for NTM bugs. As patients we have to not accept lax culture practices from our personal pulmonary physicians.

Off to lunch–more this afternoon.

Michele