Beware of “Helpful” Advice
Dr. Bruce Rubin
Posted: April 2021
As you well know, plastic bronchitis is rare, and many medical providers have never heard of it. Casting is unpredictable, often with long stretches of wellness interspersed with sudden severe and even life-threatening airway obstruction. There are also very few medical centers dedicated to the care of persons with plastic bronchitis and few effective medical therapies. This make it very confusing to try and evaluate the real effectiveness of prescribed therapies while at the same time, people with plastic bronchitis and their families are desperate for answers.
For a decade, the Plastic Bronchitis Foundation has been dedicated to serving the needs of patients with plastic bronchitis and their families. The Plastic Bronchitis Facebook page is a supportive community, so it is natural to want to turn to that community for help and advice. This advice about things like travel, referrals, insurance, etc. can be very helpful. However medical suggestions and advice based on one individual’s experience is usually not helpful and can be very dangerous.
In 2005, I started an International Registry to collect data about the presentation, associated conditions, natural history, and effect of therapies for plastic bronchitis from physicians around the world. Through this registry and collaboration with leaders like Drs Itkin and Dori, we have identified apparent risk factors for plastic bronchitis – and some of these are potentially modifiable – and have been able to evaluate some data about potential therapies that we have published in the medical literature.
Many therapies offered are not helpful and some can be dangerous. As but a few examples, asthma is a very common disease affecting about 10% of children. It does not appear to be more common in children or adults with plastic bronchitis although many patients with PB report being misdiagnosed as having asthma for years before the correct diagnosis was made. Persons with PB who do not also have asthma receive no benefit from taking asthma medications like albuterol/salbutamol or tiotropium (Spiriva) and corticosteroids that suppress the immune system can increase the risk of secondary infection. Dietary changes are often recommended and although not likely to be harmful, there is no evidence of effectiveness.
More concerning is that some patients with congenital heart disease have underwent surgical modifications of their Fontan (e.g. take down, fenestration, redo) only to find months later that this made no difference to casting. Medications like Pulmozyme (dornase alfa) are very effective in treating cystic fibrosis but increase the risk of lung function deterioration and even death when used to treat asthma, chronic obstructive lung disease (chronic bronchitis), bronchiectasis not due to cystic fibrosis! Pulmozyme should not be used to treat plastic bronchitis.
There are therapies that can be life changing in treating plastic bronchitis, and other treatments that appear to improve casting. These will be discussed in future articles on PB pages. In the meantime, avoid advice from well-meaning persons posting online and be skeptical about treatments proposed by a doctor who is not familiar with plastic bronchitis.