A huge issue with any chronic illness is treatment compliance. Did you know that in order to listed for any transplant in the United States that the patient and their caregivers have to show their willingness to comply to treatment, any treatment prescribed or recommended by your team? Yes really, if you are not going to comply with treatment then how are you going to take the necessary steps to protect that precious donated organ that they are giving you.

In PCD care we already have such conflicting treatment recommendations from experts around the world. We have a PCD specialist right here in the states that thinks airway clearance techniques aren’t needed in PCD care because the PCD patient retains the body’s natural cough clearance. There is an equally prominent PCD and Bronchiectasis expert that says airway clearance is an absolute must to ward off early onset pseudomonas and mycobacteria infections in PCD patients. The biggest thing is that there are no scientifically proven treatments, medications, diet, and or therapies for primary ciliary dyskinesia patients, not even anything FDA approved i the United States.

Now add in the internet and the world stage of other medical systems in the world and their treatments and guidelines on how they think PCD should be treated and it’s no wonder you have patients like me scratching our heads. Scratching our heads trying to figure out what we really should be doing to help ourselves. Because so and so in this support group says do A,B, C and another person says do A & C, but doing B isn’t recommended in PCD care by their governing medical body for their country. One country uses N-acetyl cysteine (NAC) and another country says absolutely do not use N-acetyl cysteine (NAC) in PCD. It’s all very confusing to the average patient, especially the younger generation.

I truly believe this disconnect is the reason there is so much noncompliance issues surrounding treatments especially airway clearance in primary ciliary dyskinesia care. I myself struggle with compliance for different airway clearance techniques. Part of me just hates them. And part of me doesn’t trust that they work. I really struggle with am I making things worse. What does anyone really understand about PCD treatments yet? These are all the things swirling through my head as. I dread the next treatment session.

My personal thoughts are that this issue isn’t going to get any better until we have better studies that answer some of these questions. And we have experts that are consistent and maintain continuity of recommendations across the board. I mean how do we expect the youth to get on board with treatments that even some PCD experts can’t even get behind. I really think that the experts and the clinicians need to meet patients where the patient is. Some of us just can’t wrap our heads around the conflicts amongst the experts and the recommendations. If we had a set of guidelines that were across the board, no expert descent then maybe more of us would be on board with airway clearance treatments and PCD treatments in general.

I think caregivers could take a page from this thought of meeting the patient where they are. It’s okay to switch up your airway clearance. It’s okay to make it fun and relaxing or both at the same time. Airway clearance doesn’t have to look the same for every single one of us. And honestly constantly pushing us to where we aren’t ready isn’t doing anyone any favors. Try meeting your PCD loved one where they are, where they are comfortable, and then slowly working up to where you want them to be. PCD is a process, it stretches out over decades, and there is no finish line. We all, patients, caregivers, and medical professionals need to relax, try to be supportive of one another, and work together to work through the process of life with PCD.

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“Here smell this. Does it smell to you?”, this is a common occurrence in my house. Or rather it used to be, because my family gets tired of it. See it’s just safer for me to just not trust anything that’s not fresh out of a box, like just opened for the first time. Ten to one if the lunch meat has already been opened for the first time, I won’t touch it again unless there is another person to decide if it’s not spoiled. I typically avoid milk that’s not fresh unopened from the store period, I hate leftovers too.

A person without primary ciliary dyskinesia may not truly understand these things, but it took one time just once getting food poisoning for me to decide that my lack of sense of smell can be dangerous to me. Did you know that repeated infections of the sinuses are thought to be the culprit behind the loss of the sense of smell in PCD patients? Researchers aren’t exactly sure why we lose our sense of smell, but the loss of the sense of smell in person has gotten a lot of attention in the post 2020 world. Did you also know when you lose your sense of smell you also experience a loss of taste or an altered sense of taste? I can’t tell you the last time I really tasted my food. This is most definitely a safety issue for those of us with PCD.

Can you imagine not being able to smell smoke as well? What if your house is on fire and you can’t see it because it’s in another part of the house? What if you can’t smell the smoke? I think that the general population doesn’t truly understand how dangerous it is to live in this world without your sense of smell. Be safe my friends!

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Can a PCD patient do too much airway clearance? The answer may surprise you. It is totally possible to do too much. The lungs are just like a muscle, and just like a muscle you can injure it by doing too much. You can also cause issues by doing airway clearance techniques improperly. So what does this all mean?

Let’s start with improper techniques. There is a reason that you shouldn’t run out an buy airway clearance devices just because they are available to buy. The patient should be evaluated by medical professionals to see if it’s safe and effective for them to do airway clearance and which devices or techniques that the patient should use. This is because not all devices and techniques are the same for every person. Some people will be unable to use certain techniques for airway clearance because of their unique anatomy, their unique over all health picture, and all their other medical issues/comorbidities. These coexisting issues can make certain techniques unsafe for the patient, and only a licensed medical physician can make that determination. So just because a certain airway device is all the rage in social peer groups, it doesn’t necessarily mean that it’s safe for you or your PCD loved one to use. You could actually injure the body’s fragile lungs using a device wrong. There is so much misinformation out there even from the manufacturers that it’s hard to determine which is the best advice. You need the advice of a medical professional who is up to date on their medical journals, so that you get the right advice. Remember manufacturers rarely update their recommendations or instructions due to FDA rules to get the devices through the regulation process. Also not all airway clearance devices are regulated, which means can you truly trust the sales information for the device. For proper trainings and demonstrations of airway clearance techniques and devices ask your PCD respiratory therapist today.

Not only is using the devices and techniques wrong an issue in airway clearance, but doing too much can be a major issue as well. You should have an airway clearance plan with your team, if you don’t have one ask for one. You want to know the plan ahead of time, and you want to understand the plan. The lungs can be overworked just like any other part of the body. If you are doing too much you could be tiring out your lungs and yourself, thereby slowing down or hindering the mobilization of secretions. Mobilizing secretions is the whole point of airway clearance. If you or your lungs are tired and overworked then you may not be getting the full benefits of airway clearance. That’s why it’s important to stick to your plan. Using airway clearance techniques, and the medications that go along with it, too close together can tire out your airways. In fact too much bronchodilators, too close together can over-relax the airways and actually inhibit getting the mucus out of the lungs and cause many other issues; like gas trapping and atelectasis just to name a few. It’s very counterproductive to overdo your airway clearance and it’s most likely going to make you miserable. Talk to your PCD team today about your airway clearance plan.

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I don’t think that most people without PCD realize how overstimulating sounds can be to a person with PCD. The cilia are responsible for moving the natural ear fluid down the eustachian tubes. In PCD the fluid doesn’t flow properly to the throat through the eustachian tubes, so as new fluid is made, it builds up in the middle ear and into the mastoid area. The build up of fluid interferes with the person’s ability to hear properly. Sometimes the PCDer will experience hearing loss making the person unable to understand and process sounds. And some days, this fluid acts like a megaphone and amplifies the sound making sounds extremely loud and unbearable. Unfortunately these impacts are transient, meaning they come and go. The patient never knows how their hearing will be from one day to the next, even one minute to the next. This transient hearing also makes getting accurate hearing tests for PCD patients near to impossible.

I can’t tell you the amount of times my ent will say my audio gram looks good, but my hearing stinks from day to day. It’s completely frustrating for me let alone my spouse. There are also days I long for quite because all the sounds are amplified and I’m over stimulated and it really affects my frustration level. If you have a little PCD person on your hands it’s often difficult to understand that sounds can be quite frightening on a daily basis, especially when the hearing ability fluctuates. Your little one’s frustration and overwhelmed feelings can be driven by their inability to cope with transient hearing symptoms and the reactions of those around them. Honestly I had that bipolar parent who couldn’t deal with my fluctuations in hearing ability and punished me for them. Please remember that if it’s frustrating for you, just imagine how frustrating it is for those of us with PCD ourselves.

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