Dry mouth (xerostomia) is basically the lack of saliva. Many health conditions or treatments of heath conditions can lead to dry mouth. Our mouths have a balance of good and bad bacteria, also know as flora, dry mouth leads to an unbalance in oral flora which can lead to tooth decay, cavities, tooth fractures, plaque, gum disease, oral thrush, and tooth failure. Today we will discuss some of the causes of dry mouth as well as some other issues related to oral care as it pertains to the PCD (Primary Ciliary Dyskinesia) patient.

Some of the causes of dry mouth in PCD patients can actually be tracked back to their medications; such as bronchodilator usage (short or long acting), corticosteroid usage, antihistamine usage, and antibiotic usage. Sinus issues caused by PCD, lead to mouth breathing thereby causing dry mouth as well. PCD patients using oxygen are also at higher risks for dry mouth. As are PCD patients who use sleep therapy devices used to treat sleep apnea.

Some other oral hygiene issues as they relate to PCD are over-brushing, improper technique when utilizing nebulizer machines, and lung flora/ bacteria. Everyone should brush their teeth in the morning, before bed, and after meals. But it’s important to avoid over-brushing your teeth. Over-brushing can actually lead to excessive washing way or brushing away the already weakened tooth enamel; this causes the teeth to become weaker and venerable to decay, fractures, and tooth failure. It’s for this reason that patients using inhaled medications, such as bronchodilators, corticosteroids, and inhaled antibiotics are asked to rinse their mouth out after use of these medications instead of brushing. Rinsing is suggested to prevent an imbalance of oral flora, which can cause thrush, and to prevent washing away already weakened tooth enamel. It’s also always good to practice proper technique when using a mouthpiece while taking a nebulizer treatments. To do this the patient should form a seal around the mouthpiece with their lips and support the nebulizer cup with one or two hands. The patient should never clench their teeth around the nebulizer’s mouth piece nor should they just use their teeth to secure the nebulizer in place with or without hands. The vibrations from the nebulizer cup and it’s mouthpiece can actually be transferred to the teeth when the teeth are used to secure the nebulizer in place or when the teeth are clenched down on the mouthpiece. These transferred vibrations can cause cavities to increase in size, tooth fractures to form, and lead to early tooth failure. Another consideration in oral care for PCD is that the bacteria in the lungs can travel over the gums, the teeth, and the mouth as we breath. These bacteria can cause the gums and teeth to become inflamed especially if there was already tooth decay present. It’s suggested to rinse your mouth out after coughing out mucus and after each airway clearance to prevent the bacteria from your lungs from entering your bloodstream via tooth decay, inflamed gums and jaw, and or tooth fractures.

Frequent dry mouth can be attributed to many aspects of PCD and PCD care. Learning and practicing things that you can do at home to help your overall oral health picture will help ward off tooth decay, cavities, gum disease, plaque, and oral thrush, tooth fractures and early tooth failure. There are also many things your dentist can do to help as well.

At home avoiding spicy or salty foods can lessen dry mouth. Be sure to rinse after inhaled medications and mucus clearance instead of excessively brushing your teeth after them. Practice proper technique with nebulizer cups to prevent tooth fracture and or early tooth failure. Keep water with you at all times to give your mouth some extra moisture that it lacks from dry mouth and mouth breathing. Water will also help you stay hydrated which can aid in maintaining the balance between good and bad oral flora. Sucking on something sour and or chewing gum is another way to help your saliva to hydrate your mouth. Utilize lip-balm is a great tool to help maintain your oral flora. And, if you use sleep therapy machines opt for a mask instead, to minimize the risks of dry mouth related to sleep therapy machines.

Even though you are practicing good oral hygiene, PCD patients can also benefit from a dental plan that you and your dentist agree on. Prompt treatment of any mouth issues that arise can ward off major dental problems in the future. It’s also suggested that lung disease patients see their dentist twice a year for cleaning. During this cleaning your dentist may suggest fluoride treatments and sealants. Sealants are another big preventative step in oral care in PCD patients. Sealants are generally applied only once a year for the general population. However for those patients with PCD, it is suggested that you get sealants applied at least twice a year.

Proper oral care is a very important, but easily overlooked, part of PCD care. Knowing the pitfalls and strategies to combat those pitfalls will hopefully lead you to a long and healthy oral history with your PCD.

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 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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