It is very hard to define typical ‘stages’ for a disorder like PCD that has such a wide spectrum of severity with some people being very ill and experiencing serious lung impairment from an early age and others doing quite well with minimal intervention. We are still in the process of understanding the natural course of PCD over a lifetime, so what we know at this point is based on patient experience. The information provided here is based on patient report and does not represent an official position on the stages of PCD. It is subject to change and may not apply to all patients. Because it is not possible to accurately describe stages at this point, we have limited it to three broad categories just to give affected individuals some idea of what to expect. The PCD Support Group in the United Kingdom has broken these stages down further, for those interested in more complete picture. Their stages can be found here:
Please be aware that, internationally, we know only a small fraction of all patients with PCD, so these observations are subject to change as we learn more.
The early years (ages 1-5) and initial school years (ages 5 to 10) appear to be challenging for many PCD patients with chronic infections of the lungs, ears and sinuses requiring frequent medical intervention and the daily need for airway clearance breathing treatments. Antibiotic therapy is often required, including the occasional need for IV antibiotics for some children.
Adolescence and Early Adulthood
While not an official finding, many parents have noted that as the child approaches adolescence, the symptoms of PCD seem to become more manageable (this is observational only and does not apply to all cases of PCD). The patient group refers to this as the ‘Cinderella period’ because, while PCD does not go away, the constant, unrelenting need to address infections seems to decrease a bit. This can be misleading, as PCD is progressive and it is still very important to maintain daily therapies to protect the lungs from permanent damage.
The ‘Cinderella period’ can last for years or even decades and most children and young adults with PCD are able to complete their educations, work, get married, start their families, etc. Daily airway clearance therapy is still critical during these years, as is prompt, aggressive attention to infections. The goal remains preserving lung function.
By the third or fourth decade of life, the ‘Cinderella period’ ends and the number and severity of lung infections increases. The organisms responsible for infections become more nasty and difficult to treat and bronchiectasis advances. Lung function declines and as the patient ages supplemental oxygen may be required. Ear problems can recur and many adults with PCD require hearing aids. Many patients report disability due to lung impairment by the fifth decade of life, some much earlier. Patients with advanced lung disease from PCD may consider lung transplant as an option. Transplants have been successfully performed in patients with PCD, including those with situs issues.