Preventative care is essential in PCD to slow the progression of the damage done to the body as a result of PCD. Lung tune up, a phrase coined by the Cystic Fibrosis community ages ago, has proven to be an effective preventative treatment in CF. As PCD has no specific treatments, a lot of PCD treatments are borrowed from CF research and CF treatments. Lung tune ups are basically a treatment plan that last from four to six weeks where the patient gets intense airways clearance at least four times a day, IV and inhaled antibiotics with the antibiotics being guided by the patient’s previous and current culture results, and lots of additional rest balanced with activity to aide in airway clearance. The thought behind lung tune ups is to give the patient a boost with antibiotics and increased airway clearance before the patient becomes exhausted and depletes their energy reserves to fight infections. This boost will hopefully aide the patient in keeping up with their peers. Lung tune ups are great for those patients who are less than compliant with their treatment plans, and younger patients who’s treatment plans rely on others to preform.

Lung tune ups historically were carried out only on an inpatient basis, in North America, back when airway clearance techniques were solely done by hand. Caregivers had a hard time sticking to at least four treatments a day requirement of lung tune ups, because the caregivers arms would tire out. IV antibiotics or intravenous antibiotics had to be administered by licensed nurses, as the medications are caustic to the veins and require frequent replacements of peripheral IVs. Inhaled antibiotics had to be closely monitored and adjusted on a patient by patient basis. IV medications required close doctor monitoring and adjustments as well. All these things could be accomplished easily in a traditional hospital setting. Think of lung tune ups as a sort of boot camp for your respiratory system, it takes many people to accomplish the goals of a lung tune up.

With the invention of airway clearance devices, such as the vest airway clearance machine and hand held percussors, coupled with research into cross contamination and infection control; lung tune ups began to shift focus in the modern world. Now a days most specialist shy away from doing lung tune ups on a complete inpatient basis and started leaning towards completing tune ups at home. Some patients to this day are still treated for a tune up as before on a total inpatient basis for the complete duration of their lung tune up; especially the children. However most young adults and adults can begin their tune ups with a brief three to six day hospital stay and then complete the tune ups in the privacy of their own homes. IV antibiotics can be delivered via a PICC Line or a Port-o-cath while being monitored by a home care nurse with the guidance of the patient’s doctor. Airway clearance machines all but eliminate the problem of fatigue during tune ups for the caregiver. There is also less risk of the patient being exposed to more germs and bacteria that they would be exposed to during an inpatient stay. Lung tune ups at home are also more cost effective for the insurance companies and the patient in question as well. Other patient advantages to doing lung tune ups at home is increased rest, decreased stress, patients can remain social, and patients can go to work or school as they are able to.

As we look to the future in regards to lung tune ups, some facilities and specialist are shifting towards what is best for the patient; especially considering the emergence of more and more antibiotic resistant super bugs. Airways clearance machines are also becoming more common place in the everyday treatment of other respiratory diseases other than CF and PCD. These advances are helping to eliminate the need for lung tune ups anywhere but the patient’s home. Inhaled antibiotics have shown success at treating lung bacteria at its source instead of relying on systemic treatment via IV antibiotics. And the convenience of machines to complete airway clearance make patients less at the mercy of others when it comes to getting their airway clearance done. Hopefully future advances in respiratory care will lessen the burden on PCD patients and their caregivers.

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PFTs or Pulmonary Function Tests and the results of those tests are one of the most confusing things for a patient to understand; even if they do the tests quite regularly. PFTs can be as simple as one breathing maneuver repeated three times in a row, often called Partial PFTs or Basic PFTs. Or PFTs can be more complex testing, often referred to as Full PFTs or Pre and Post PFTs. So what actually are the PFTs? The PFTs are called spirometry, lung volume, and diffusion capacity. The spirometry, lung volume, and diffusion capacity tests can be done before inhaled medications, after inhaled medications, or both before and after.

Preparing for your PFTs is as important as the tests themselves. Be sure that you understand the test or tests that you’ve been scheduled for, and when and if you should change how and when your inhaled medications need to be taken prior to the tests. To enable you to breathe your best for the testing be sure not to consume heavy meals as these will make you uncomfortable during the testing phase. Also wear lose clothing to insure you can breathe deeply and comfortably. All of these precautions will help you to succeed in doing your absolute best for your PFTs.

The simplest and most widely used PFT is spirometry. Spirometry uses just one breathing maneuver repeated at least three times. The patient sits in a chair, ensuring to sit tall and with both feet on the ground in front of them. The patient places a mouthpiece into their mouth and breathes normally for a few breaths, then they take a deep breath in (deep as they can), then they exhale as fast, as hard, and fully as possible until all the air in their lungs are empty, and then they inhale a breath. Spirometry tests are often done pre bronchodilators, post bronchodilators, or both pre and post bronchodilators. Doing the test both pre and post bronchodilators can tell your doctor if bronchodilators help your lungs or not. Spirometry testing measures both the amount of air being exhaled and the time it takes to exhale that amount. The testing is done three times or more to ensure the results are reliable and reproducible.

Spirometry measures many different volumes, how much air is moved; and flow rates, how fast the air moves. The common measurements in spirometry are as follows. Forced Vital Capacity also known as FVC; is the volume of air exhaled from full inhalation to full exhalation. Forced Expiratory Volume in the First Second also known as FEV1; is the volume of air that a patient can forcefully blow out in the first second of the FVC. A decrease in your FEV1 compared to normal values from a group of people who are your same weight, height, sex and are nonsmoking individuals may indicate a decrease in your flow rates. Ratio of FEV1 to FVC also known as FEV1/FVC, is calculated by dividing a patient’s actual FEV1 with patient’s actual FVC and reporting the result as a percentage. A patient’s FEV1/FVC can help determine what type of lung disease or lung damage that the patient has or may have. Peak Expiratory Flow, Peak Flow, or Forced Expiratory Flow Maximum also known as PEF, PF, or FEF Max; is the fastest flow rate reached at anytime during the FVC usually occurring near the beginning of the patient’s forced exhale of the FVC. Maximum Mid-Expiratory Flow also known as MMEF or FEF25-75, is the flow rate in the middle of the patient’s forcible exhale. MMEF or FEF25-75 is a very sensitive measurement of air flow obstruction in those patients with mild disease. If your tests are ordered with pre and post bronchodilators you will receive two sets of results one set for pre bronchodilators and one set for post bronchodilators. If the pre bronchodilators test shows obstruction, but the obstruction disappears during post bronchodilators test this is usually indicative of the patient having some form of asthma.

Another group of PFTs that helps your doctor gage your lung health are Lung Volumes. The three most commonly used tests to gage lung volumes are as follows. Nitrogen Washout; done by breathing oxygen normally while exhaled gas is collected and analyzed for residual nitrogen. Helium Dilution; done by breathing in a gas mixture of helium and oxygen during normal breathing. Plethysmography also known as the Body Box, the most accurate lung volume measurement technique; done while the patient sits in a box (enclosed chamber) and preforms very small panting maneuvers. The separate volumes of air measured during lung volume testing are as follows. Total Lung Capacity or TLC; the maximum amount of air that a patient’s lungs can hold, which is measured at the very top of inhalation. Vital Capacity or Slow Vital Capacity also known as VC or SVC; the maximum amount of air that can be exhaled during normal and slow exhalation after a patient has inhaled to their fullest. Functional Residual Capacity also known as FRC; the amount of air left in the lungs after a normal exhalation. Residual Volume also known as RV; the remaining air in the lungs after the patient exhales all the air they possibly can. Tidal Volume also known as VT; the amount of air that is inhaled and exhaled with each breath the patient takes, basically normal breathing while at rest. Inspiratory Reserve Volume also known as IRV; the greatest amount extra air that a patient can inhale after normal inhalation. Inspiratory Capacity also known as IC; the amount of air that a patient can inhale after exhaling a normal breath. Expiratory Reserve Volume also known as ERV; the greatest amount of air that a patient can exhale after normal exhalation. Lung volume testing can help your doctor decide if you have a restrictive lung disease or an obstructive lung disease.

And lastly your doctor may order Diffusion Capacity also known as DLCO; which measures how well gases move from the lungs into the blood, like oxygen for example. There are several ways to do DLCO with the most common being the ten second single breath hold. The patient is asked to take a single breath while inhaling a mixture of harmless gases, they then hold that breath ten seconds, then exhale that breath and as the patient exhales the machine takes a sampling of that exhale. The machine then compares the inhaled and exhaled gas concentrations to give a reading of how well oxygen moves from the patient’s lungs to their blood. This helps the doctor to determine if there is damage where the oxygen and the blood meet in the patient’s air sacs also known as alveoli. Higher than predicted DLCO is rarely cause for concern. Whereas a lower than predicted DLCO can indicate a large variety diseases that your doctor will need to sort out what the actual issue is by using other testing and other readings from your PFTS.

In PCD, PFTs are used regularly to gage and monitor the overall picture into the patient’s lung health. The PCD Foundation recommends that PFTs be conducted for surveillance purposes at least two to four times a year. PFTs can help your doctor see emerging trends in your lung function, see drastic changes in your lung function, and overall help monitor your lung function and lung health over long periods of time.

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When you are in your late twenties several questions keep popping up and very few have anything to do with being chronically ill, this is simply because these questions are asked by all of us at some stage.

You have likely had several jobs. Chances are good that you are in or have experienced a long-term relationship. You live in a new city, enjoy travel, and have a slightly more grown-up outlook on life compared to when you were 21, you might have started a family or have new friends. In your late 20s, early 30s, there are lots of new things in one’s life.

We often divide life into three sections: career, relationships, and health. Most people place these things into separate boxes. Unfortunately, when you are chronically ill with a disease such as PCD, these boxes are less separate and represent more of a Venn diagram. Some sections overlap more than others, and they can infringe on each other even if you do not want them to.

Growing up, normal children are told that they can have any career they can dream of when they leave school if they work hard at achieving their goals. Children that grow up with PCD, however, have a harder time choosing their career path as they need to consider their health. We include questions such as how close are we to your nearest specialist? Is the environment we work in conducive to having cilia that don’t work that well, or work at all for that matter? Will our bosses or co-workers understand that we need to take it slow on occasion and that we don’t simply get the flu, we most likely get pneumonia and end up in hospital a couple of times a year. Please understand, I am not saying that we are limited to an office-bound career, but being an archaeologist in the Egyptian desert is going to be a little more difficult if you PCD than being a lawyer or teacher in the city.

PCD does not shout out “here I am” for all to see, often those of us who suffer from it, do so quietly and nobody knows. We deal with our symptoms in such a way that people think we might be asthmatic with an unpleasant cough and runny nose. There is no scarlet letter for all to see. Which, unfortunately, means that many people do not understand why we cannot go out to clubs whereas, far as you can see, is a haze of cigarette smoke or interact with large groups of people as they might give you something that your system cannot deal with. Not to make light of the recent COVID-19 pandemic, but those of us with PCD found the concepts of wearing masks and maintaining social distance not that strange, as we automatically do some version of this in our daily lives.

No matter where life takes us, we find that in a relationship we ask, is this person going to be able to cope with the trials that having a partner with PCD presents? Sometimes we are very lucky, and we find that person that is willing to stand by us through the ups and downs. The doctor’s appointments, the physiotherapy sessions, the maintenance treatments at home and the mental struggles that go with being chronically ill. Some of us struggle to become parents, again we ask can our partners cope with that.

When we get to our late 20s, those of us who suffer from PCD know our bodies well, there is an intuition we develop with regard to our health. We know when that cough is nothing major to worry about and when it’s time to get our doctors involved.

The hardest part of being a young adult with PCD is making sure that the disease does not define who you are. This is not impossible, but it does take a substantial amount of hard work. It is essential that people you surround yourself with understand that you are not going to break the moment something goes wrong, you are stronger than you think.

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