From their very first breath of life primary ciliary dyskinesia (PCD) patients begin their life long battle against bacteria and debris that they breathe in. The lungs are perhaps the most environmentally exposed internal organ in our bodies. As a PCD patient ages the bacteria that causes infection mutates and becomes harder and harder to treat. This physiopathological progression leads to numerous hospitalizations to treat infections and stabilize patients with PCD with aggressive airway clearance and intravenous antibiotics. Oftentimes these hospitalizations come without warning during a normal pulmonary followup or following an acute onset of exacerbation symptoms.

In my personal experience with my PCD there is a very real need to have your bags packed and ready to go on a moments notice with life necessities packed way inside them. I most always have my “Go-Bag” ready and waiting. I routinely rotate items in and out of my bags to keep things fresh and updated. My go-bags take the stress of unexpected hospitalizations off of me and off of my Mister. He doesn’t have to worry about rushing home to throw together items I might need, or think that I will need for my hospital stay. I also don’t have to worry about figuring out how to get my things if I'm hospitalized. Nor is there any last minute dash home to retrieve my things, myself, before reporting to the hospital admissions office. Go-bags are a great tool to help relieve some of the chaos in my PCD life.

As I’ve gotten older into my forties and my bacteria has mutated to near antibiotic resistant. My medications have changed to harder to get medications, and Ive even had a port-a-cath placed for IV access. These harder to get medications may not be available at your hospital. And if you are like me my port isn’t a power port-a-cath, since it’s main use is antibiotic therapy. Believe it or not hospitals do not routinely carry non-power port-a-cath needles for port access. I always carry extra port needles in a small zipper pouch, in my purse, at all times for emergencies.

Another issue for aging PCD patients is our use of non-mainstay inhaled antibiotics for our breathing treatments. Last year I had a surgical procedure and was admitted post procedure due to post operative respiratory distress to a major metropolitan hospital, in the Twin Cities in Minnesota. Very much to my surprise their hospital pharmacist asked my Mister to return home to retrieve my home supply of Colistin, because the hospital pharmacy didn’t have access to get any for my breathing treatments. I also have a ton of medication allergies because not only was I gifted with PCD from my folks; but my mother’s sulfa, sulfate, and sulphites allergy was passed on to me. That leaves a ton of allergies to medications in pill form. Something I can’t take orally in pill form due to anaphylaxis, can be given intravenously in liquid form without the preservatives often found in pills that I am allergic to. Upon hospitalization routinely I am hit with, “We don’t have your exact medication, but here is this and it has the same active ingredients. So it will work the same.” Ummm, in my situation it’s just not the active ingredients that send me into anaphylaxis it’s the inactive ingredients as well. So it is not uncommon for me to have to bring my inhalers or mediations that I know the hospital is not going to have. Then there is airway clearance with breathing treatments at the hospital, so I take my own vest garment for maximum comfort. I do not take the air generator part of my vest system though, you know the actual machine; actually my home hospital frowns on taking your machine portion in to their facility due to their cross contamination policies.

Other areas to consider when deciding your essentials for your bag are entertainment, snacks, and drinks. PCD patients are often faced with contact isolation restrictions while hospitalized due to the poor understanding of cross contamination of bacteria risks. And then like my situation, now I consistently culture antibiotic resistant bacteria, so I’m always in isolation. I need things to keep busy, and I know what I like, but it is hard when being inpatient to go out and retrieve things to keep me busy. I’ve had nine day inpatient stays where I never even flipped on the hospital television, thanks in part to my iTunes library on my iPad. Do not forget to pack your devices’ power cords. An extension cord, or two, as well as a power strip might be a good addition to your bag. My current home hospital recently instituted a sugar ban on all hospital food services; no sugary drinks of any kind. Try getting a juice from your unit nurse when your sugar is low, from your lack of desire to eat due to being acutely sick. It is a nightmare! Now I pack my own drinks and snacks. Snacks because let’s face it, a slice of cheese and a pack of two saltines when you are hungry outside of normal room service hours is a pain. And if you like coffee, don’t forget your own sugar packets and cream; good luck finding those too. I've been known to sneak over to the Starbucks on my hospital campus a time or two while dragging an IV pole along behind me. And to pull off that kind of visit to the local Starbucks, I'm obviously not in a hospital gown and slippers either. Nope, I pack my own lounge clothes and pajamas. They are the first thing that I change into out of my street clothes on admission. You can virtually wear your own clothes while in patient and even for most procedures as determined by your attending physician/hospitalist/floor doctor. I even wore my own lounge clothes into my last port placement procedure. The interventional radiologist who placed my port said as long as I was comfortable, he was fine with no hospital gown. You will find a lot of places work with you, and if you don't ask, they don’t bother unless it strictly goes against hospital policy.

It is because of the aforementioned things that I now actually have multiple go-bags for the hospital; one for is my actual go-bag with the basic essentials to get me through those first days, one is a medical go-bag with a my medicine, vest garment, port care needs, one is a craft/entertainment/snack/drink go-bag, and one is a long haul go-bag to get me through those long hospital admissions. Ultimately the number of go-bags that you need really depends on your personal preferences, however you should have at least one go bag packed and ready with your essentials for a short stay at all times. It would be a great habit to get into, just throwing that go-bag in your trunk on your way out the door for all clinic visits.

Recently I asked my PCD community the following question, “What do you think is essential for your hospital go-bag?” Here are their answers; names have been omitted to protect patient privacy. Some closely related responses may have not been included.

- Cell phone charger and hair ties!!!!!

- Kindle for me ♥️ cosy slippers or slipper socks, dry shampoo.

- In all seriousness, we actually take our vest. Our hospital only had wraps and my PCDr is autistic to boot. Anything we can keep familiar the better. And he loves HIS vest. 🙂

- Vest, pajamas, clean underwear, and his computer and phone.

- Mine brings his stuffy. 100% of the time. And Hogwarts blanket.

- For my 17yr old, vest- Pillowcase from home, loose comfy sweats, slides, chargers, Chap stick & crossword books!

- Cell phone and charger, underwear, toothbrush and paste, brush, blow dryer, slippers, housecoat, book, shampoos and that kind of stuff (I don’t want to pay for theirs.)

- PJs, blankets, snacks, cellphone and charger, deodorant, toothbrush. That's what's essential for me.

- For our 7 year snacks are a huge thing! I make sure to pack granola bars, apple sauces, usually Goldfish crackers & Bear Paws. My son is always always hungry & so tiny. The meals usually provided by the hospital are totally insufficient. Other than that we bring his backpack with iPad, charger, favorite stuffy & blankie & a book.

- Favorite blanket, pillow, pj's, phone, laptop, earbuds, chargers, extension cords for chargers, lotion, (bathroom items), chapstick, cute socks, colored pencils, coloring book, favorite insulated cup, candy, chips, favorite snacks, favorite stuffed animal.... menus to order food from restaurants.

- So we don’t we bring a ton...

But a must is vest, slippers, blanket, pillow, and extra underwear. (Since my son is younger he’s been through a few pair of undies with antibiotics... and it’s not pretty). Sometimes it’s nice to have snacks in my purse. But the most are above.

The hospital we typically stay at tend to provide a lot of the distractions for us.

- My daughter always has her PCD blanket, fuzzy socks (the best), Aloe vera kleenexes for that sore nose. hand sanitizer, a cuddly, doesnt matter your age! Snacks essential oh and music.

- Their own pjs. Favorite stuffed animals, snacks, and their phone of course.

- Chargers and cords! Pens and a small notebook. Contact lists of important friends/family just in case you need to have someone else make a call for you. A cozy blanket for the parents it is always cold overnight!

- Snacks and the Xbox are how we survive extended hospital stays. I also second slippers!

- Underwear

- Ok so most people would classify this as essential but for me it is. About 14 years ago when I was initially diagnosed and I lost my lung, my little brother bought me a teddy bear. I was in the ICU at that time and he was too young to come into the ward. We have always been super close. This was his way of being there for me. This bear has since then gone with me to hospital with me every time and reminds me that no matter how bad things get I am never alone. Phone charger

- A book!! Always a book! Or if you're not a big reader, some kind of portable entertainment

- For me, nail polish. When I'm super sick, I don't care about my nails. But as soon as I start feeling a little bit better, painting my nails cheers me up and makes me feel a little less gross, since I inevitably have greasy hair and limited shower access... So, whatever your thing is that makes you feel put together and happy.  Look better, feel better.

- Also, your go to comfort food.  I make sure I've got a variety of snacks to satisfy whatever I'm craving.  I even take my own tea bags so I have unlimited herbal tea. 

- Slippers or flip flops that you can easily put on when you get out of bed and not step on the hospital floor. 

- I also hate wearing a hospital gown and will always just wear pajamas or comfortable workout clothes unless I absolutely have to wear a gown for a procedure.  I pack tank tops (easy to put on with a picc line) and zip up hoodies/ sweaters that have oversized sleeves I can easily put on over an IV or picc line.  Layers because I constantly get hot, cold, hot, cold from the medications (and/or fevers). 

- That’s such a good question. Comfortable pj's  for if chilly or hot and good book is a must for me.  Oh and some biscuits.  Oh and nice smelling soap.  I bet I think of more later lol.

- phone charger!

- I always take my own comfy clothes-leggings, pjs, layer type shirts for the temp changes and easy telly/iv line use, slippers/fuzzy socks, coloring books, word finds, iPad with movies, music, and books, ear buds, and chargers. My own cozy blanket, window clings for feel of home. And I always have my normal shower routine stuff (good lotion and body wash, make up too!) Because toward the end of hospitalization it's a good sign if I tried more. 🤣🤣🤣 I always take sanitizer wipes and baby wipes. Mints for when I am nauseous. I'm sure I'll think of more.

- Extra-long phone charger, eye mask for sleeping with too many lights on, and spares of any medical bits that the hospital isn't likely to have. And a one-page summary of my meds and recent medical history so I don't have to recite it over and over again when I'm short of breath :)

- Headphones, phone charger. Maybe the notebook. Clothes and my glasses but i barely put them off

- Comfy pjs, your own pillow and fuzzy blanket, things to keep you busy (I go crazy when stuck in bed). Take your own vest if you have one it will save $$ off of your hospital bill.  Snacks and drinks because hospital food is awful.

- Yes- chocolate!  I'm very lucky, my visitors usually ask if they can pick up anything for me to eat on their way to the hospital.  Usually tacos or skittles.  :D

- Note pad and pens, lotion (so dry and all the hand washing) word search or crossword puzzle books, an uplifting quote, warm socks....

- Slippers! I always bring slippers

- I always load plenty of films and box sets on my iPad. I always wear clothes never in PJ’s only to sleep.

- Nice hand wash, speaker as in my own room and gives my ears a break. My favorite

- hot chocolate books I never get round to reading (who am I kidding)!!!😊

- My tablet, My pillow (I wish,I don't take it cause of germs) hate hospital pillows and because of all the coughing, my own pads the hospital ones suck.

My husband, although it's hard getting him to fit in the bag. No joke my hub stays with me every night.

      Whatever your needs, we hope this gives you an idea on items to tuck into your go-bag to take away one more item of stress fromliving the PCD life. Remember that needs change over time, and some items do not handle longterm storage in your go-bag. Other things are just not practical to always keep in your go-bag, so create a list of missing items for quick reference during the rush to pack for a hospitalization. Stay safe and “Turtle On” my PCD peeps!

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Cilia are thought to play an important role in organ placement during the embryonic stage of development in human beings. Nodal cilia are responsible for the establishment of the lateral positioning of organs as the embryo develops. These nodal cilia are very similar to motile cilia found in the respiratory tract of the body. Without proper movement of the nodal cilia, organ placement becomes random throughout the thoracic and the abdominal cavities of the body.

There are three main descriptive terms used to describe the position of both 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. 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 primary ciliary dyskinesia (PCD) 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 2018 study published 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, 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 in complete mirrored image from their normal positions; situs solitus. In situs inversus 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. Situs inversus rarely involves other heart health issues as well as any other medical issue; with the rare exception of PCD. Sometimes situs inversus is called situs inversus totalis but they both technically mean the same thing. However old school medicine used to term stius inversus totals as a complete flip left to right of the body axis, and situs inversus was used to differentiate only a complete flip or either the abdominal cavity or the thoracic cavity. Depending on which doctor you ask today this mistake is commonly repeated. Situs inversus is supposed to be a complete mirror image flip left to right in both the thoracic and abdominal cavities, not just one or the other.

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. Levacardia is a descriptive term used to describe organs displacement 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. Situs ambiguous patients tend to have severe cardiac abnormalities, spleen abnormalities (asplenia or polysplenia), intestinal rotation, and or 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.

When a person also has primary ciliary dyskinesia and some form of organ displacement they are said to have PCD with situs inversus, or PCD with situs ambiguous. In days gone by PCD patients who also had situs inversus were said to have Kartagener’s syndrome. Kartagener’s is an outdated term that describes only one subset of PCD. This outdated terminology still persists in today’s society because the name usage has been in medical journals for over a century despite the forward movement of research that has advanced the medical field’s understanding of cilia. PCD patients with any other form of organ displacement other than a complete flip or mirror image actually have PCD with situs ambiguous, including those with multiple spleens and or those with a missing spleen. For more information on the terms used to describe organ displacement in correlation to PCD please visit the PCD Foundation’s article, "What’s in a Name? A Brief History of PCD Terminology".

Researchers are not sure to what extent that nodal cilia actually play in the embryological stage of human development and why certain patients with ciliary dyskinesia have organ displacement and some do not. Hopefully as our technological understanding of the cilia and the diagnostic tools used to examine cilia advance this and other answers will reveal themselves.

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 your 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.

Be sure to visit us next week for another Topic Thursday!

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PCD (primary ciliary dyskinesia) awareness is an essential part of finding a cure for PCD. Awareness is also a difficult topic in the world of PCD. How do we raise awareness without showing our weaknesses as patients? Do we really want the world to know what we go through? How do I share my family member’s PCD and protect their privacy? Is spreading awareness about PCD necessary, or is it attention seeking? And finally why is PCD awareness so essential in finding a cure for those with PCD? Let us start by examining these aforementioned questions.

So how do we raise awareness without showing our weaknesses as patients? You know the old saying that “Beauty is in the eye of the beholder”, who’s to say the same thing can’t be said about weakness as well. When we share our PCD stories, concerns, frustrations, or our PCD path in general it is not weakness to share the good and the bad. Actually it takes real courage to show others our vulnerabilities, our fears, and our trails right alongside of our triumphs. Sometimes our greatest strength is actually our own perceived weaknesses. So share your weaknesses right along side of your triumphs and embrace your journey with PCD, because the person you may help might just be the person behind you on the PCD path.

And do we as patients really want the world to know what we go through? This is a question that I ask myself every single time I decide to share something about my journey with PCD on social media. Everywhere I look there are always these nice rosy stories about PCD triumph and virtually no stories on the lows of PCD. In fact google the question, “Will PCD kill me?” You will find all these nice neat packages and quips about how a person can live a rosy long life with PCD just like the normal population. There is always attached to said articles on the web, that this is true for patients with PCD as long as they were diagnosed early in life. This, in my opinion, is absolutely wrong. I was diagnosed at a very early age, treated at a very early age as well, and my PCD path has been filled with not so rosy details. However there seems to be the need to paint such rosy pictures of the PCD life, but it is anything but rosy to those of us in trenches. Awhile ago, while doing some researching, I ran past a presentation given on PCD in association with a PCD Foundation meeting that said that the median life expectancy in PCD was just forty-six. Forty-six years of age, and that was a presentation given around 2006 if I remember correctly. Based on that revelation I then went in search of what researchers believe the current average life expectancy in PCD is in the year 2019 and the number that I came up with from an anonymous source places the current average life expectancy at just sixty-seven. My source doesn’t want to go on record at the present time. Part of the reason is that these numbers are just rough estimates based on incomplete data and anecdotal evidence only. There are several PCD patient registries being established and put together all over the world. Once the data from all of the accredited PCD Centers of Excellence are put into the registries the answers to some much sought after questions should emerge. These patient registries have the capability to change the PCD world in ways we can’t even begin to fathom right now. Our very understanding of PCD may change overnight. This new understanding of PCD can only happen by sharing everything; our complete PCD experience the good, the bad, and the ugly. We as patients have to be willing to share everything.

You might be asking yourself how do I share my family member’s PCD and protect their privacy? In today’s age of social media this is a very tough question. I’m personally thankful that social media wasn’t around when I was a child. My family overshared everything. I wasn’t given the right to object until I was eighteen. Everyone knew everything, and I despised my family for it. In a way, this very issue continues to shape my relationship with my family to this very day. Bullying was a big thing back in my day too. I believed and still believe that if my family wouldn’t have freely told everyone on the planet, including perfect strangers on the sidewalk every tiny detail that my bullying experience would have been much different. I’m not just talking about educating my teachers and staff about my PCD. Yes teachers and direct care people need to know. However, they didn’t need the nitty gritty details of my experience. Like let’s say, my antibiotics where giving me a yeast infection. These adults who where told this, then freely talked about my yeast infection in front of my peers, my peers parents, other teachers, and so on. They really didn’t know when to stop, they really didn’t care about my privacy, and they never took my feelings in to consideration. I was just food for gossip with everyone. People really need to stop and take into consideration a minor child’s right to privacy. This is especially a big thing when dealing with a special needs child. They deserve privacy and the right to choose. So how do you share a family member’s journey with PCD? Ask them, run your idea or ideas for sharing by them and get their approval, and mostly involve them in your awareness efforts; because it’s their life and they will be the ones who have to deal with the fall out when they come of age.

Is spreading awareness about PCD necessary, or is it attention seeking? I often get asked if I’m oversharing my PCD experience. Is my endgame attention seeking or is it really awareness? But here is the thing, yes it is a fine line between spreading awareness and attention seeking. The only way to create awareness is to seek out attention, but is it the right kind of attention. I don’t want sympathy, that is not why I share my PCD experience. I want people to know and understand the PCD experience so that they are better equipped to deal with their PCD loved one. And yes sometimes that loved one I am talking about is me, when the person reading my awareness postings is a family member or friend of mine. By helping others understand the PCD experience I hope to ease the burden of PCD on my fellow PCDers. If people do not know what we go through, then how do we expect them to help those of us with PCD. How do we expect them to care about the fact that cures for a rare diseases are most oftentimes funded by the friends and family members of those with the actual diseases. If they don’t know, if they don’t understand, and if they don’t care how are we ever going to find real treatments and ultimately a cure for primary ciliary dyskinesia?

And finally, why is PCD awareness so essential in finding a cure for those with PCD? Well I think I’ve pretty much covered that in the last paragraph. Let me just add that besides helping advance research by helping with funding issues, we need to raise awareness so that other people actually hear the name; primary ciliary dyskinesia. PCD needs to become a household name, just like CF is, if we ever hope to find a cure; let alone fund a cure. We need to get medical students, medical researchers, and the pharmaceutical industry interested in PCD. We want to bust the myth that PCD is a mild disease that most grow out of; which is truly false by the way. But these medical and pharmaceutical people actually still believe these untrue things, because some old medical journal says these untrue things are true. These medical and pharmaceutical people aren’t interested in keeping up with the emerging data that is proving that PCD is anything BUT a mild disease. I had a friend go speak in front of a group of medical professionals at a medical conference a few years ago. The conference attendees where shocked to learn my fellow PCDer and friend required and received a double lung transplant due to PCD, because to their knowledge from school PCD rarely resulted in the need for a lung transplant. Well I can name a dozen PCDers who are post-double lung transplant and just over twenty PCDers who are currently either on the transplant list waiting their transplant, or are currently going through the transplant evaluation process before being listed for transplant.

PCD awareness is an essential part of finding a cure for PCD, because PCD is so widely mischaracterized in severity and outcomes. We need to raise awareness to raise the funds to actually help ease the burden of PCD on not only myself, but my fellow PCDers as well. How do we raise awareness effectively and safely? We accomplish this by keeping the name PCD or primary ciliary dyskinesia out there and in everyone’s faces. We want interest in PCD not only in the medical and pharmaceutical industry, but in the insurance industry and in the everyday world. Keep spreading PCD awareness people! Tell your truth! Help us find and fund a cure for primary ciliary dyskinesia.

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Nebulizer machines turn liquid medication into a fine mist that the patient then inhales. Proper care and feeding of your nebulizer and it’s components like nebulizer cups, tubing, spacers, masks, mouth pieces, and even the nebulizer’s air intake filter are extremely important. Improper care can lead to an increase in lung infections and or acquiring bacteria that you have not previously cultured. It’s important to remember that the patient will be breathing in the mist that is created inside the nebulizer cup. So a clean nebulizer and nebulizer components are a must to maintain airway health.

There are several ways to approach daily cleanings of reusable neb (nebulizer) cups. One is to use a fresh clean reusable neb cup for every treatment of the day. So if you need four breathing treatments a day, you will use four different and clean neb cups a day. After using each neb cup for the day, simply set each used neb cup aside for cleaning after your last treatment of the day. With this method it is safe to use the same neb cup for each of your medications; assuming you follow the path of bronchodilator first, followed then by your mycoltic (hypertonic, pulmozyme, etc), and lastly your inhaled antibiotics. Remember bronchodilators first, then mucolitics, and lastly your antibiotics; if you don’t do them in this order it is important to do them each in separate neb cup. Its also important to remember never to mix the medications altogether at one time either; refer to your health care provider for your care team’s desired medication order for your unique health picture. The other way way to approach daily cleanings with reusable neb cups is to thoroughly clean, disinfect, and dry after each and every single breathing treatment. And the last option for daily cleaning is, if you are using a disposable neb cup, you simply throw it in the trash after each use. Disposable cups are meant to be used one time only.

Daily cleanings are an important part of a patient’s respiratory health; as the patient will be breathing in a mist created by the neb machine. Daily cleaning recommendations really boil down to what is the patient’s respiratory disease. In CF (Cystic Fibrosis) and PCD (Primary Ciliary Dyskinesia) it’s important to go the extra step of disinfecting every single day to prevent the spread of germs and bacteria. So reusable neb cups should be cleaned first, then disinfected, rinsed, and finally air dried every single day.

To clean reusable neb cups, they should be taken apart piece by piece; though do not remove any valves or the valves off of the face masks and or mouthpieces. Fill a bowl with warm water and dish soap, place nebulizer parts and masks or mouthpieces and any spacers into the solution and let soak for fifteen minutes. After fifteen minutes swish the parts around in the solution, then remove and put in clean sterile water or rinse with clean sterile water. Pull out the pieces from the clean sterile water and shake off the excess water from the pieces; then immediately follow with disinfecting the pieces. It is important to note that the use of antibacterial dish soap or white dish soap in the cleaning solution is NOT appropriate for some neb parts from various manufacturers; please refer to the manufacturer of your nebulizer and it’s parts.

There are multiple ways to disinfect, depending on your neb manufacturer. Please refer to your neb manufacturer on which is best; the cold method or the hot method. The hot methods for disinfecting are as follows; boiling for five minutes (Pari neb cups are one of the few manufactures who say their neb cups can be boiled, so use caution.) Another hot method is microwaving for five minutes (Again Pari brands are only one of the few neb cups that can be microwaved “depending on the particular Pari model”.) The third hot method is putting them in a dishwasher and washing at one hundred fifty-eight degrees for approximately thirty minutes (Again refer to manufacturer, as Pari neb cup are currently the only neb cups rated for hot methods of disinfecting.) Finally the last hot method is an electric steam sterilizer, like a baby bottle steamer. This is a very popular method for disinfecting neb cups in the PCD and CF communities. Hot methods of disinfecting are only recommended for neb cups (mainly the reusable Pari neb cups, but refer to your manufacturer’s recommendations.) It is important to note that hot methods used on equipment not intend to be sterilized in this manner can actually damage the neb parts; including all masks, mouthpieces, spacers, and neb tubing. The cold methods are as follows; soak in seventy percent isopropyl alcohol for five minutes, or three percent hydrogen peroxide for thirty minutes then rinse with clean sterile water. No matter which method that you use, it is important to remember to follow up both methods hot or cold by rinsing the neb parts in sterile water. You can buy sterile water from your pharmacist or make it yourself by boiling water for five minutes. Then be sure to allow the neb parts to air dry completely on clean towels/ paper towels prior to their next use. Remembering to not to put the face masks or mouthpieces face down on the drying surface to prevent the fibers of the paper-towels/ towels from collecting on the mouthpieces where they can then be breathed in during the next treatment. Neb tubing that connects the neb machine to the neb cup can be wiped after each use with a clean wet cloth or disinfecting wipe; never submerge neb tubing. The machine itself can be wiped as needed with disinfecting wipes between individual treatments. The air intake filter of your machine should NEVER be submerged in any liquid.

Is it time to feed my nebulizer, and what does that even mean? Feeding your nebulizer is basically exchanging your reusable parts for new reusable parts. Reusable parts to neb machines are not meant to be permanent; they wear out, start collecting bacteria, and then the patient can become infected with the bacteria that is growing in their reusable neb parts. Exchanging out reusable neb parts is another essential step in maintaining your respiratory health. Reusable neb cups, masks, mouthpieces, spacers, and tubing should be replaced every three months, unless it is a Pari reusable neb cup system. Pari rates their reusable systems at six months instead of the industry standard of three months. Your reusable neb parts should also be exchanged for new reusable parts if they appear worn, damaged, or do not seem to be working properly or like they were when you first put them into use. Nebulizer machines should be cleaned with disinfecting wipes as needed or at least at the end of every day. If your neb machine has an air intake filter, the filter should never be cleaned or disinfected to extend the life of the filter; instead those air intake filters should be replaced every six months to maintain your machine’s level of performance. Air intake filters that appear wet or spongy should be replaced immediately even if the six month are not up yet. Neb tubing that appears to have moisture inside the tubing should also be immediately replaced, no matter how little the tubing has been used.

Proper care and feeding of your nebulizer machine and it’s parts will extend the life of your machine, maximize treatment times, and protect your respiratory system from unwanted germs and bacteria. For more detailed information on your particular nebulizer system, including which reusable parts are appropriate for your exact nebulizer system, please speak to your particular care team. A quick note of caution, due to the powerful nature of some nebulizer machines, use of any incompatible reusable neb tubing or parts can actually cause those parts to melt. For instance, only Pari nebulizer parts should be used with a Pari nebulizer machine to prevent the neb tubing from melting onto the Pari neb machine itself.

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