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Lower Airways (Lung)
The goal of treatment in PCD is to slow the progression of disease. One way to do this is to be aware of the baseline state and monitor for any variations from baseline that might indicate an infection is present or the disease is becoming more advanced. There are several routine surveillance practices that can help with this, including sputum culture, pulmonary function testing and imaging/bronchoscopy as indicated.
PCD is a progressive lung disease. Progressive lung disease potentially means progressively worse bugs, so routine 'surveillance' cultures are recommended. The recommendation from the European Task Force on PCD is that routine sputum cultures be collected at least every 6 – 12 months when healthy and every time there is an active lung infection. Additionally, people with PCD are at increased risk for unusual infections from less common bugs like non-tuberculous mycobacteria (NTM). Collectively, these bugs are known as ‘acid-fast bacilli’ and they require specialized cultures. The current recommendation from the GDMCC research group is that people with PCD should have acid-fast bacilli (AFB) cultures annually and as symptoms indicate.
Pulmonary Function Tests (PFTs, aka ‘spirometry’)
Pulmonary function testing or PFTs (aka 'spirometry') is a surveillance technique that measures how effectively your lungs are performing by tracking the amount (volume) and speed (flow) of air being inhaled and exhaled. To perform PFTs, the patient is asked to blow into a mouthpiece while a computer records the force of the expelled air, how long it takes to fully empty the lungs and a number of other values that give an overall picture of lung function . The two values of most interest to doctors treating PCD are the FEV1 (forced expiratory volume in 1 second), which measures how effectively your lungs are able to expel a large volume of air in the first second of exhalation and FVC (forced vital capacity) which shows the total amount of time required to expel all air from the lungs.
PFTs have proven to be a very reliable tool for measuring and predicting disease progression in conditions like cystic fibrosis, asthma and COPD. Their utility in PCD is not as clear at this point, but the European Respiratory Task Force on PCD still recommends that PFTs be done every 3-6 months.
CT scans are the most reliable way to assess for early changes in the airway that might suggest bronchiectasis. The current recommendation is to consider a baseline CT scan to assess level of lung involvement when the diagnosis is first made. However, there is an elevated risk of radiation exposure with CT scanning, so this is something you should discuss with your physician.
Regular x-rays pose a much smaller radiation exposure risk, but they are not particularly good at picking up bronchiectasis until it is quite advanced. They are an important tool for monitoring infections like pneumonia.
Bronchoscopy is a procedure that allows for direct visualization of the airways by means of a lighted scope passed down the trachea and into the lungs. It is normally done under anesthesia for children and using local sedation for adults. Bronchoscopy can be exploratory, diagnostic or therapeutic in nature, and allows for samples of tissue and mucus to be collected for pathology, visualization of the walls of the airway to diagnose conditions like bronchiectasis, removal of mucus plugs or foreign bodies and the collection of fluid that has been used to ‘wash’ the airways (called ‘lavage’) for analysis.
Upper Airways (Ears and Sinuses)
PCD-related conductive hearing loss is very common in young PCD patients and routine hearing tests should be performed to assess level of loss. More permanent forms of hearing loss may occur in older patients, so testing is important as PCD patients age, as well.
Cultures of the drainage from ears or sinuses can sometimes be helpful for determining a course of treatment.