The ACTs or Airway Clearance Techniques of PCD (Primary Ciliary Dyskinesia) are used to help transport mucus from the smaller airways to the larger airways. Once in the larger airways the mucus can be coughed or huffed out of the body. PCD patients should do one or more daily ACTs, depending on their care plan. ACTs can be done in conjunction with other treatments like nebulizer treatments; such as bronchodilators, mucolytics, and nebulized antibiotics. However, it is suggested that nebulized antibiotics be done after ACTs are completed. ACTs move mucus to help you breathe better, reduce lung infections, and improve PFTs (Pulmonary Function Tests.) ACTs can be done by almost anyone, the important thing is to actually do the ACTs. So what are some of the common ACTs?

Let’s start with the most basic ACT; coughing. Coughing clears mucus with high speed airflow. However because of the nature of PCD and it’s effects on mucus in a PCD patient sometimes coughing alone doesn’t clear the mucus. And coughing a lot can tire the patient out, make the patient more short of breath, and make the patient feel much worse. Huffing is a type of cough that is not as forceful and not as tiring. Huffing involves taking a breath in and actively exhaling it. Think; huffing onto a mirror to fog or steam it up.

Chest Physical Therapy, also known as Postural Drainage & Percussion or P&D, uses gravity to drain the mucus from the smaller airways to the larger airways, where it can be coughed up. This is done by another person using cupped hands with percussion to the patient’s chest to loosen the mucus from the airways. This is done as the patient lays in various positions that allows gravity to pull the mucus along the airways; where it can be coughed or huffed out.

The Airway Oscillating Device (AOD) or Oscillating Positive Expiratory Pressure (Oscillating PEP) uses resistance to open airways, and vibrations from the device shake the mucus loosening it from the airway walls. The resistance allows air to get behind the mucus and that air then helps to move the mucus from the smaller airways to the larger airways, where it can be coughed or huffed out. Common AODs or Oscillating PEPs are the Flutter™️, the Acapella™️, the Cornet™️, and the Intrapulmonary Percussive Ventilation (IVP)™️. With their lips firmly around the mouthpiece of the device, a patient exhales completely through the device several times, followed by coughing or huffing. This cycle is repeated as prescribed by their care team.

The High Frequency Chest Wall Oscillating Vest or Oscillator uses an inflatable vest attached to a machine that vibrates the vest at high frequencies and inflates the vest to various pressures. This technique works by compressing the chest in and out to create airflow which loosens the mucus from the airway walls. Vibrations then carry the mucus from the smaller airways to the larger airways. And, every five minutes during the treatment, the patient stops the machine for a few seconds to cough or huff out the mucus. Vest settings are determined by your care team, and vary from facility to facility and patient to patient.

Positive Expiratory Device or PEP uses airway resistance to open the airways and get air behind the mucus, loosening it and moving it from the smaller airways to the larger airways; where it can then be coughed or huffed out. A PEP System includes a mask or mouthpiece attached to a resistor machine; like one of the settings on the Metaneb™️. The patient breaths in normally and exhales a little harder against resistance.

Active Cycle of Breathing is designed to get air behind the mucus making it easier to cough up and lowers airway spasms. There are three phases of Active Cycle Breathing. First is Breathing Control, the patient gently inhales through the nose and out the mouth. Second is Chest Expansion Exercise, the patient inhales deeply and holds their breath for three seconds. This gets air in behind the mucus to loosen it and moves it from the smaller airways to into the larger airways. The third and finally phase Forced Expiratory Technique, the patient huff coughs to move the mucus out of the larger airways and out of the body.

Autogenic Drainage (AD) means self drainage. AD uses varied forms of airflow to move mucus from the smaller airways to the larger airways. It also has three phases. First is Dislodging Mucus, the patient takes in sallow breaths to unstick the mucus from the walls of the small airways. Second Collecting Mucus, the patient collects the mucus by holding their breath for three seconds. And the third phase Clearing Mucus, the patient uses hard huff coughs to move the mucus out of the airways. AD takes hard work in the beginning, but most people adapt quickly. However; it’s important to note that AD is NOT recommended for ages eight years old and younger.

Physical Fitness, the last ACT we will discuss today, can be especially important in people with PCD. Even though the patient may have trouble breathing and tire easily, Physical Fitness can help in a number of ways. It can impact the patient’s overall health, provide more energy, improve lung function, and help the patient spend more time with others out and about. Even when hospitalized a patient should get as much movement in as possible to help them maintain their level of fitness. Patients should be active NOT uncomfortable; exercising three to four times a week, doing aerobic exercises that last more than ten minutes is recommended. As is; one to two days a week the patient should include resistance training, such as weightlifting. It is recommended that adult patients get twenty minutes of exercise a day, and children get sixty minutes of exercise a day. Above all a PCD patient should exercise only to the extent that they are physically able to based on their overall health picture, and with the guidance of their care team.

This is just a brief overview of some of the ACTs of PCD. ACTs can vary based on the patient’s disease, the patient’s care center, or even the patient’s country of residence. Although PCD patients’ ACTs can vary, the PCD Experts all agree on one thing; the BEST ACT is the one that the patient will actually do. The experts suggest mixing up ACTs daily, weekly, or monthly depending on the patient’s interests. Keep it fresh, keep it interesting, but most importantly do the ACT that is of interest during the patient’s scheduled time for ACTs.

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 Cilia are thought to play an important role in organ placement during the embryonic stage of development in human beings. There are three main medical terms used to describe the position of thoracic and abdominal organs in the human body. They are Situs Solitus, Situs Inversus, and Situs Ambiguous. These terms can be further broken down into several others to further explain exact placements of certain organs or combinations of organs. But today we will focus on these three terms; Situs Solitus, Situs Inversus, and Situs Ambiguous.

According to NORD (National Organization for Rare Disorders) approximately fifty percent of PCD (Primary Ciliary Dyskinesia) patients have some form of organ displacement in the human body. The other approximately fifty percent of PCD patients without organ displacement, as well as the rest of the human population without organ displacement, fall into the Situs Solitus category. A recent study published this month by the U.S. National Library of Medicine cites that out of the fifty percent of people with PCD who have organ displacement, twelve percent of those patients have Situs Ambiguous, also known as Heterotaxy Syndrome, leaving the remaining thirty-eight percent in the Situs Inversus category. It’s important to note here that while fifty percent of people with PCD have some sort of organ displacement, only twenty-five percent of people with organ displacement actually have PCD as well. So what does Situs Solitus, Situs Inversus, and Situs Ambiguous actually mean in terms of organ placement and or displacement?

Let’s begin with Situs Solitus. Situs Solitus is the normal position of the thoracic and abdominal organs. This means the heart is on the left side of the body along with the aorta, bilobed lung, single spleen, stomach, and the systemic atrium of the heart is on the left side of the heart along with the cardiac apex. On the right side of the body is the inferior vena cava, trilobed lung, liver, gall bladder, and the pulmonary atrium is located on the right side of the heart.

Meanwhile in Situs Inversus the thoracic and abdominal organs are reversed or mirrored from their normal positions (Situs Solitus). In the most common situation of Situs Inversus, Situs Inversus Totalis, there is a complete transposition of all abdominal organs and the heart is on the right side (known as Dextrocardia). Dextrocardia literally means right-hearted. This common situation of Situs Inversus rarely involves other heart health issues as well as any other medical issue; with the rare exception of PCD. Levacardia on the other hand is where the heart remains on the left side of the body (normal position) but other organs are reversed or mirrored including in the abdominal cavity. Levacardia usually also always involves other structural defects of the heart which can be life threatening at birth and may require immediate medical intervention.

Then we come to the most confusing; Situs Ambiguous, also known as Heterotaxy Syndrome . Situs Ambiguous is an organ displacement abnormality that involves both components of Situs Solitus and Situs Inversus inside the same patient. These patients exhibit abnormal organ arrangement across the left-right axis of the body. They have neither the normal, usual, reversed, or mirrored placement arrangements. Situs Ambiguous patients tend to have severe cardiac abnormalities, spleen abnormalities (Asplenia or Polysplenia), intestinal rotation, and malabsorption of the abdominal organs. The mortality rate is high in Situs Ambiguous mainly due to the complexities of the malformations, in particular the severe cardiac malformations.

So which Situs are you? Only your physician is qualified to clarify that answer for you. There are many imagining options out there to help your physician determine organ placement; MRIs, Ct-scans, Ultrasounds, and X-rays. Unfortunately the older population and even some of the younger population today may be walking around with organ displacement and not even know it. Their physicians may not even be aware of it either. How can this be you ask? Back in the day when organ displacement was seen on an X-ray, it was often dismissed as the X-ray was backwards and no further investigation was initiated. That is a less common occurrence in modern medicine today. Unusual findings are almost always investigated by a patient’s care team. However it’s possible some organ displacements are still missed to this day, especially if the patient has no other health issues that warrant x-rays or other health interventions or investigations.

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The United States PCD Foundation held its very first town hall meeting on 30 March, 2020 with one hundred and sixty nine attendees from the PCD patient groups. The virtual town hall panel consisted of North American primary ciliary dyskinesia experts Dr. Adam Shapiro of the Montreal Children’s Hospital, Montreal, Quebec Canada; Dr. Amjad Horani of the St. Louis Children’s Hospital, St. Louis, Missouri; Dr. Pamela McShane of the University of Texas Health Science Center, Tyler, Texas; and Anne Griffiths of the Children’s Minnesota, Minneapolis, Minnesota. The major theme of this virtual event was COVID-19 in the context of the PCD patient. The overall consensus of the panel was that the safest place for PCD patients and their immediate families is in their homes, unless the PCD patient needs respiratory support.

The virtual town hall got off to a start with introductions of the town hall panel members, who then took turns answering questions submitted by patients and their family members via the Zoom teleconference platform. There was a brief overview of the COVID-19 infection and its possible implications to the PCD population. To date there isn’t enough data that would indicate the potential of COVID-19 complications to the patient with PCD. The lung damage being seen post COVID-19 infection in recovered patients appears to be fibrosis like damage from the distinct type of pneumonia being seen with COVID-19 infections. Whether this will translate to lasting impact on post COVID-19 patients and their FEV1 scores remains to been seen. So far there are no known reported cases of COVID-19 in a patient with primary ciliary dyskinesia in North America today. To the panel’s knowledge this is true for Europe as well. The panel consensus was that this might be a good sign, but honestly there just isn’t enough relevant data at this point.

When pressed for recommendations for the PCD community, all panelists were in agreement that the patients need to limit contact until this event (pandemic) is over. Patients and their immediate family members should shelter in place for at least the next four weeks, with no one in or out of the home. If someone has to go for essentials, they should deliver essentials to the porch, clean and disinfect them, shower and change clothes, while washing the clothes they wore out. Patients and their families should avoid drinking and eating after one another. As well as avoid takeaway/take out and food not prepared inside the bubble of their own home. Take out poses a risk of infection from the food service industry employees and those that the employees comes into contact with. Patients and their families should severely limit the number of people who come and go from the home to virtually no one, including non-essential nursing services provided to the home. Stay in your bubble, stay in your homes, for at least the month of April and even the month of May. June and the months beyond June will have to be determined based on how the coronavirus ultimately unfolds in North America. Even once the peak of the virus has passed and various areas of North America start to lift their shelter in place orders, the PCD community will need to be very vigilant for at least the next twelve months or more. Prepare for things to stretch on and the possibility that the PCD community will be impacted by COVID-19 much longer than the general population. Remember there is no magic bullet for this. Well the magic bullet is actually to STAY HOME.

If your PCD family includes essential employees that must work, care must be taken to protect the PCD family member, including isolating them from others in the home. If the family member works in health care, that family member should not be performing duties that involve direct patient care. Performing clerical work from home instead is the best alternative for these families. PCD patients who work in the medical field should be working from home where possible or just not working. Families are encouraged to talk to their PCD care providers to obtain letters for employers to get work accommodations and or assistance.

The topic of shared parenting was brought up, and the general consensus was to limit travel between households for PCD children at this time. Shared parenting households are encouraged to work together to find solutions to limit their child’s exposure to as few people as possible. Keep their bubble small. This doesn’t mean that children should be kept from their other parent. But it does encourage parents to work together and find beneficial solutions to limit their child’s exposure to others. In researching this topic briefly, we’ve seen that this topic has been at the heart of several emergency court hearings. Even though most courts are currently closed or operating on emergent basis, court officers are holding virtual hearings to help parents navigate this unprecedented time. There are currently no States in the US who are granting a suspension of visitation rights solely because one parent is an essential employee or healthcare worker. The courts are suggesting that parents work together to limit an at risk child’s exposure to other people in the best interest of the child. And that the parent retaining custody in a shelter in place or quarantine situation makes extra effort to allow telecommunications with the other parent as often as necessary, and that after the limitations are lifted to grant extra visitations on a case by case basis. Parents in the above described situations should make every effort to work with one another and each other's attorney as needed.

PCD patients are encouraged to continue all their therapies and not to discontinue anything or add anything without speaking directly to their care team. The panel members encouraged patients to keep up with their sleeping, eating, and exercising habits. This gives the PCD patient a good overall pulmonary edge or stamina if the PCD patient was to contract COVID-19. Patients should cough into a tissue, then bin it (throw it in the trash), and wash their hands. Each member of the panel said continuous hand washing is a must. Wipe down all surfaces, handles, and regularly clean the bathroom. There is indication that an infected person can shed the virus though their stool for a month or more. Sharing a bathroom with a suspected COVID-19 patient is not an option. Even people with mild illness can spread COVID-19 for many weeks. The panelist also said lightly wiping down delivered packages would be a good idea as well; although being overly obsessive about it is not necessary. PCD patients should treat all symptom flares as a PCD flare until the symptoms are proven otherwise.

PCD patients are encouraged to keep their stamina up and to stick with low risk activities. Now is not the time to end up in an emergency room with a broken limb. The emergency room is the last place a person with PCD needs to be right now. PCD patients should utilize all their protocols at home as much as possible. If your treatment protocols at home are not working contact your PCD team. It is important to note that any aerosolized treatment option can spread COVID-19 through the air if the patient receiving the aerosolized treatment is infected with COVID-19. It is for this very reason that almost no emergency room in North American is currently giving breathing treatments in the emergency rooms. Emergency rooms have switched to inhalers instead. COVID-19 patients are being treated, where possible, in zero pressurized rooms at facilities across North America. Individual facilities are working hard to come up with ways to give patients breathing treatments in their own facilities while at the same time protecting all other patients and medical personnel. If a PCD patient ends up hospitalized for non COVID-19 issues, their access to breathing treatments will most certainly be impacted. Stay home, your treatments are at home. This issue with aerosolization and COVID-19 will shape the way facilities handle breathing treatments, CPAP usage, BIPAP usage, and ventilator usage for the foreseeable future. Stay at home in your bubble, it is the safest place for a patient with primary ciliary dyskinesia. We are in this for the long haul North America.

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For Rare Disease Day 2018 we asked the PCD (Primary Ciliary Dyskinesia) community, “What are your things NOT to say to a person with PCD?” Here are their responses. Some closely related responses have not been included in this list.

- My ENT said, “We need to get your hyper-secretions in your sinuses thinned out, so your cilia can do their job and move the secretions out of your sinuses.”

- My Respiratory Therapist said, “Breath in through your nose, so that your cilia can filter out germs and bacteria as well as moisten the air going into your lungs. Moist air helps the lung cilia work optimally too.”

- “Blow your nose.”, my daughter hates that.

- The gym teacher says, "Come on you can do better than that". I say let me tape a straw to your mouth and nose and you run. 🤣

- "You sound like you have a bad cold" which is similar to "Wow what a horrible cough". And it's always the same people over and over again which is when I get annoyed, one time is not harmless. I don't look sick. I don't expect everyone to know my background, but when you've said it 8 times in 3 days wake up.

- “If you're sick, you should stay home!", but then we’d never go out. 🙄

- “Well, at least it’s not CF.” (True, but minimizing the real long term medical effects and feelings of PCD is really obnoxious. I am happy my son doesn’t have CF, but it’s no cake walk having a child with PCD).

- When a provider says, “PCD? What’s that?”

- “I bet my doctor can cure you.”

- “Just take some Honey.”

- “Eat this magical bush or drink this tea.” Lol

- “Do you smoke?”

- So many variations of; “You should lay off the smoking, that’s what you get for smoking, etc”

- “It’s not like you had surgery.”

- From doctors " What do you mean that medicine doesn't work how do you know?"

- My pharmacist lectured me that too much ciprodex would cause the cilia to not work....after I told her they didn't work right to begin with.

- “Just use XYZ essential oil/herb/cleanse and you will be as good as new!!”

- “But you look so healthy!”

- From the school regarding my kids, “Well, we just need to make sure they are here more often and not just staying home with a sniffle.”

- “Can you just stop blowing your nose?!”

- “You go to the doctor too much!”

- "Do you want a cough drop?”

- “Breathe in through your nose and out through your mouth" (the last is said all the time during exercise. I'm like how the heck do I do that when the exercise makes my nose run??)

- Direct quote from my (former) chiropractor... “You know, if you have a miscarriage, it’s God’s way of cleansing the body and it could cure your PCD.”

- “You should really see a doctor about that cough.”

- “I have asthma too...” Um I wish it was just​ asthma..

- I get, “Are you sure it’s not asthma?”

- “And I see here he must have a little asthma?” 🙄

- “I’m sure she’ll grow out of it. My daughter was sick a lot when she was that age and she’s fine now.”

- “Do you think she’ll grow out of it?” Me, “No she’ll never grow out of it.” Them, “but maybe she will!” 😡

- “When he gets better...”

- When I was in school I played soccer and my team had to run miles but my coach had me run laps at my own pace while my team took their route around the sports complex for safety reasons and to be near my inhaler. Members on my team would always say "You're not sick! You're just too lazy to run!"

- Lately the one that gets me is "When are you having kids?" Since most don't realize the miscarriages and complications related to PCD and pregnancies.

- “You're not getting younger, you need to start your family!". At the time, I had a fridge full of hormones that I was injecting into my stomach every day, among other things (blood draws every other day, more hormones in the butt, lots of ultrasounds). Some people who get pregnant easily just don't get it.

- "Try a gluten free diet, it will change his life"

- My husband's old boss could not understand how sick I was and would always tell him "It's just allergies!”

- From doctors, “Are you sure you just don’t have really bad asthma? Ummmm.  Let’s see...  if my ears and sinuses are also my lungs, then maybe?

- We have one child with PCD. Someone told me we should have had more, in case, you know, something happens to her.  😒

- My personal pet peeve: "You should cut out dairy. My kids got ear infections too until they cut out dairy."

- “Oh it can’t hurt to try xyz...” Well it certainly has not changed things at all. I cut out all dairy. No change.

- “Just go to (insert holistic Dr here) because your sons on too many antibiotics and needs to get off of them and his inhaler.”

- Every new doctor, “Let's run xyz test/scan/blooddraw just in case." Let's not, we've ran them all 3 times and he's only 5. It's a diagnosis even if you have only ever heard of it.

- People tell me all the time that she gets sick because, she needs germs. “It won’t kill her.”🤬

- “Let them play in the dirt!” is one of my personal favorites.

- “Are you contagious!”

- I get.... “Oh he’s sick again? Wasn’t he just feeling bad. I’m sure as time passes and he “grows “ things will be better!!”

- "Yeah I have that sinus stuff too. It’s bad. Just take a hot shower. That will help!” 😡😡 ugh

- "It could be so much worse!" I am obviously thankful it's not worse, but coming from someone who has no clue what she and we go through it's totally annoying.

- "How is your daughter doing...is she better?  Yes she does have days that she feels pretty good, but is she ever "better"....nope...not unless they've heard of a cure that I haven't...🙄

- I had a nurse who didn't read her file ask, "Why are you calling us for just a fever and stuffy nose?"

- “So did you give this to him?”

- “So now that you know genetics diseases run in your family you should not have any More kids!” 😤

- “You should put her name on the transplant list now!  Then when you need it, your name will be in there, at the top.”  🤦‍♀️ That’s not how the system works....nor is a transplant an easy cure-all, nor does everyone qualify for a transplant, etc....

- “You need to be vegan!”

- “Your body is too acidic; that’s why you are sick all of the time!  And have rheumatoid arthritis as well.”

- “If you just gave up all animal products, you would be so much better off!

- “You probably wouldn’t be so sick if your mom had breast fed you”

- “At least he doesn’t have Cancer!” Really. 😑

A PCD family member sent us this statement, and we think it is a fitting way to end our posting for Rare Disease Day 2018. “I think all of these things reinforce how necessary and vital the PCD community is. People DO NOT understand what it's like to live this life. So the importance of a community you can lean on through the hard days and celebrate the victories with can't be understate.”

Happy Rare Disease Day 2018! Are you wearing your blue jeans today? “Hope it’s in my genes!

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