Podcast: Sinus and CF

Transcript

Voice over: Welcome to the CF Strong Podcast. CF Strong covers the successes and challenges faced by those living with cystic fibrosis. You’ll hear first-person stories, conversations with health professionals, friends and partners. Just a heads up, guests may share their personal views about treatments and health management, but please remember, this is not medical advice and you should always follow the advice of your clinic team regarding your health.

Sarah: Hello and welcome to the CF Strong Podcast. We have a special episode for you today. We’re talking all things CF and sinus, and we have some wonderful special guests to help us with that conversation. I’m going to introduce them now and then we can jump straight into our chat. Here with us today is Dr. Catherine Banks, an ear, nose, and throat surgeon with a particular expertise in cystic fibrosis. We also have Jen Hauser, regular listeners will be familiar with Jen. Jen has more than 20 years experience working as a CF physiotherapist and is the president of CF Physio. We also have Sam Lefo regular listeners will also be familiar with Sam as a regular host of the CF Strong Podcast, and Sam also has CF. Thank you all very much for joining me here today for what we hope will be a really interesting and important conversation. So I think what we’ll start with is probably a very, very broad question, Catherine, I’m going to direct this one to you. Just what are our sinuses? What are they, where are they? What do they do?

Dr. C. Banks: Okay. Right. So the sinuses are in our face and we have on each side four key sinuses. The sinuses themselves at ear filled spaces that are housed in the facial bones that they’re named after. So you have the maxillary sinus, which is below the eye and known as your cheek sinus. The frontal sinus is obviously above the eye in your forehead, the ethmoid sinuses, which extend in a high nasal region between the eyes. And then right at the back is this phenoid sinus. And there’s paranasal or paranasal sinuses is what we call them. And so there’s four on each side. So there’s eight in total. And what’s interesting is they are essentially unique, much like a fingerprint. Everyone has got slightly different sinuses. And so it’s really important for ENT surgeons to, you know, get imaging so we know exactly what’s going on within the sinuses because they are unique to each patient.

Cystic fibrosis is interesting in terms of the sinuses because they are what we call hypoplastic. So they don’t develop in the same way that patients without cystic fibrosis they tend to be a lot smaller. So the axillary sinuses are smaller and often the frontal sinuses are absent. And the function of these sinuses is really debated. You know, we ascertain that they probably decrease the weight of the face. Imagine if you just had solid bone in your face, it’d be really heavy to walk around trying to hold that up all day. They increase the resonance of the voice. They provide a crumple zone to protect the vital structures, namely the brain and the eyes. And then they insulate the sensitive structures from rapid air temperature fluxes, so hot and cold air. Importantly, the sinuses humidify and heat, the inspired air. They are also part of our innate immune system in the sense that the mucus that is produced can trap foreign particles and it can be expelled. So in a nutshell, that’s the sinuses.

Sarah: They are so much more complicated than what I perhaps had thought of them as I think.

Sam Lefo: Technically, yeah.

Sarah: Yeah. Sam, I’ll start with you. Perhaps you can talk to us as someone who lives with CF, what your experience with sinus has been like.

Sam Lefo: Yeah, it’s actually an interesting question because along with many other things to do with CF. When you get asked that question, like, what’s your experience with it? You kind of don’t really know how to explain it because you’ve only ever known what it’s like to have CF. So to me, I have with my sinuses more so now than when I was younger, is definitely noticed that there’s something not quite right with them. Actually I’ve recently been to an ENT specialist and they’ve gone in and said to me that actually there’s not a lot right up there, to be honest. So I am due to get it checked out some more and possibly get surgery. But one thing is definitely it does a feel of like a constant nasal drip in some cases. Difficulty breathing through the nose as well is quite common.

I don’t snore, I don’t think. So that’s one positive, but again, it’s kind of hard to determine what’s normal and what’s considered to be actually a sinus disease because it’s all I’ve really ever known. I was told when I was younger, I had enlarged polyps when I was going through my early stages of CF. But since then it is obviously gotten a lot worse. I am probably due to get some form of surgery in the coming months because according to my ENT specialist, it has gone a quite bad.

Sarah: I think that’s a really interesting point, Sam, that you make about not knowing what is ‘normal’ and I’m using quotation marks here. And what is something that you should follow up? And I guess that’s, that will hopefully be an important part of the conversation that we have today. Jen, just to bring you into the chat now what sort of symptoms commonly present to you as a physio when someone comes into to a clinic and that might sort of think make you think this is probably something we need to investigate?

Jen Hauser: Thank you for that question. Really important that it reflects then what Sam says. Sinus issues are very under-reported in the CF population. I think it’s often because individuals experiencing any symptoms from their sinuses either disregard it because they think that just part of CF and I’ll just have to roll with that. Lots of other priorities for their health. Obviously the lungs tend to get up there on the priority list. Then if the right questions aren’t asked in clinic, then it will get missed that they may not think it’s needed to be raised or maybe it’s not even CF so they won’t raise it there and then forget to bring it up later. The triggers for me is if someone presents with saying that they’ve got this irritable cough, particularly at night or for first thing in the morning if they’re saying that they’ve had a (inaudible 00:07:46) smell in, in terms of sense of smell or they’re doing lots of throat clearing when they’re talking, usually indicative of that postnasal drip.

Also if they’ve had recurrent chest infections that just do not seem to be responding to usual care and I start to think there something else that’s feeding these lungs and we are not treating that effectively. But yeah, as I said, often patients won’t hear any of the obvious symptoms and then if you ask the right questions, you might start to then discover that they actually have facial pain, they’ve been having headaches, they might even get referred pain to their jaw or changes in their hearing. Those sorts of symptoms can be seen in the clinic. I often think for someone, and you might be able to reflect on this as well. But I often feel when I ask patients questions, they don’t realize that there is a symptom there because you just get used to it and you live with it every day.

So it does almost seem more normal. So I definitely am a big believer of patient reported outcome measures ’cause It’s a really nice way to get a back line for someone and then later on down the track, getting them to repeat it and they can either say, “Oh, I didn’t actually even realize that was a problem for me.” And then we can actually address it and that can then lead to improved quality of life, quality of sleep with everything.

Sarah: Catherine, just to come back to you, I guess, you know, part of this conversation is we’re talking about how people might not think that a symptom is a symptom and there is, you know, this idea of common symptoms is probably a bit of bit false, but maybe you can talk to us a little bit about what you look for and when people with CF should perhaps be following up.

Dr. C. Banks:  I think that’s a really good question and I agree both, Sam, you sound like a lot of my patients and Jen, exactly what you were saying is just spot on. And the interesting and illuminating thing is that I’m an ear, nose and throat surgeon and I am trying really hard to educate my own people, you know, my own colleagues, that we actually have to be very active and ask the questions. Because we know that for patients who have cystic fibrosis, a hundred percent of them will have inflammation on a CT scan. So it’s like almost universal. And then around about 80% of them will fulfill the criteria of our international sort of consensus statements on what is chronic rhinosinusitis. Yet only 10 to 15% of patients will actually self-report symptoms. But if you ask them, you’ll pick up way more.

So you’re picking up greater than 70%. So the bottom line is that we do have to be active and we have to do the proms get everyone involved in asking the questions about whether or not you have sinus symptoms. The issue is that we diagnose chronic rhinosinusitis based on symptoms. So you’ve got to have nasal obstruction or nasal drainage issues. So Sam you’ve got — You mentioned both of those, and then you should have facial pain or pressure. Now in the cystic fibrosis population, they often don’t have facial pain or pressure. Then the other one, and it’s a big one in cystic fibrosis, is hyposmia. So decreased smell, and that’s important for malnutrition and all the other problems that can be caught up with patients with cystic fibrosis. So those are the cardinal symptoms.

Then in children it’s the cough. So we swap out the smell and we say if they’ve got a cough, and if you have that for more than three months, then your symptoms that you’re describing fulfill the criteria for cystic fibrosis. And then we have a look up your nose and we look for signs of inflammation. And Sam, you mentioned polyps, which is one of the signs, but we also look for swelling of the tissue, which we call edema. And then we look for purulence. So, big infected drainage coming out of the drainage pathway of the sinuses. Like I said, on a CT scan, it’s almost universal. Every cystic fibrosis patient is going to have signs of mucosal thickening on their CT scan. So it is really important that we all get the message out there and become very active about asking our patients what are the symptoms, and then using the prompts to record our symptoms and track that. And then, like Jen, you were saying, I think when the lungs start to go off and there’s recurrent infections for us that is a red flag and we need to be way more active in terms of what we can do for patients in terms of medical management, surgical management.

Sam Lefo: What could your long-term effects of sinus disease be? And is it different from say a quote unquote normal patient and a CF patient?

Dr. C. Banks: Definitely. The cystic fibrosis chronic rhinosinus patient is a very unique cohort. Because we now are really getting down to endotype in our patients with chronic rhinosinusitis, and we can differentiate all the inflammatory mediators and the cytokines that are driving their inflammation. But with the cystic fibrosis cohort, they’re very unique because it’s a really broad spectrum. And depending on your phenotype, you may, although you have the signs of chronic rhinosinusitis, you don’t actually have, you don’t report any symptoms. So you’ll get your scan that’s mucosal inflammation, but there is just — You’re not complaining of any symptoms. And so, for those sort of patients, you just watch and you educate to let them know. And then you’ll have other patients, for example, who just have severe polypoid or changes complete nasal obstruction and it can even alter the formation of the facial bones because their polyps are so bad.

So those patients obviously we’re going to be a lot more aggressive in terms of what we can do and what we want to do. So I think to answer your questions we need we need to be involved. But not every patient needs to have something done. We can wait. If we do wait, it’s about educating. If we decide to go in and operate then — Like I guess your relationship with a respiratory physician, I tend to think of my patients even from when the fact they’re really little, they’re going to be my patients for the rest of my life and I’m just going to take them through the journey because I know I can’t cure their sinuses, but I can certainly get control of their sinuses. And if you get control of their sinuses, I truly believe that you can get control or better control of the lungs.

Jen Hauser: Can I jump in there? Because I absolutely agree, Catherine. The point of the sinuses being united airways with the lungs is they’re all part of the same system. I think we are so passionate about being proactive about the management of mucociliary clearance in the lungs. I think it is only out of respect to the sinuses that we should do the same for them because as Catherine said, there is mucus in the sinuses, there is inflammation, there is cilia, and it’s probably not working just like it isn’t working in the lungs. And if we’re preventative in our approach and proactive, then I think we can really hope for positive outcomes. And so, from a physio perspective, that is why I bring up the question and put it in our screening and I’m hoping that we can progress to actually having it accepted as part of annual screening. And no different to our annual reviews where we do annual bloods and we do, you know, baseline chest x-ray or CT scan of lungs or lung function, what screening can we do for the sinuses so we can get that baseline and manage proactively.

There isn’t a lot of literature out there unfortunately, though, to support my passion for, for looking at inhalation therapy as a preventative form. But I certainly know, in terms of supporting the ENT specialists with prescription of any of the sprays or sinus douches, massive fan of sinus douches and so are the patients, it’s very rewarding with the massive plugs that you can get out with a sinus douche. Then looking at other inhalation therapy. And although there’s not robust evidence and it’s certainly, I’m not aware of much since 2016. The main’s team over in Germany has done some work looking at pulmazyme versus isotonic or hypertonic saline solutions and also looking at antibiotics because we have to remember if there’s inflammation and mucus up there, that’s going to get infected just as the lungs do.

So when Catherine was talking about, you know, that drainage and having secretions sampling that there’s been some work over in the UK and also in a group in Sydney who’ve done sampling from the sinuses and found that it’s isolated different pathogens so than what’s been in the lungs. So if we’re madly treating the lungs with what we’ve isolated in sputum, but not actually fighting what’s happening in the sinuses, then we are pushing uphill with that.

Sam Lefo: I think you made every person who’s CF listening a little bit scared adding one more test on our annual reviews.

Jen Hauser: I know, and Sam, this is part of the problem as well with the treatment of it. There’s this fantastic options of parisinuses that do vibration with the nebulizer as you stick it up your nose. Amazing ’cause it then vibrates and pulsates all through the various different cavities in the sinuses, but it’s another treatment. That’s what we have to be really mindful of when we are prescribing these treatments. I think if the symptoms are bad enough, people will do it, but —

Sam Lefo: Definitely.

Jen Hauser: I fear that unfortunately when the symptoms are bad enough then we’ve missed some of that opportunity. But if surgery’s been needed, then I think being really proactive with the inhalation therapy after that so we can maintain the benefits. Just to share one little tip or trick that has no evidence behind it, but it does not add any extra time to treatments. If you are doing an inhalation therapy for your lungs and you’re not combining it with an airway clearance device attached, then breathing out through your nose with your breath out, I don’t know how much of the medication we’ll get there, but maybe some of it will we can sort of be doing some targeted treatment that way maybe.

Sam Lefo: Yeah. Okay.

Jen Hauser: But yeah, it is, it’s the burdensome nature. I think Sam and I totally respect that with everything else that’s being thrown on top of lung treatments and it’ll be a changing space with CFTR modulators as well. If we think of how they’re impacting the lungs, I’m really hopeful that the same will happen with sinuses and we can see those changes in that surface of the sinuses and maybe they won’t be as big a problem.

Dr. C. Banks: Yeah, I agree. I think the CFTR modulators are really changing, you know, how the sinuses are looking and to a point where I would — If someone’s going to go on CFTR modulator, I’ll just hold off. If their sinuses need to be addressed and just to have a look at them and follow them up post CFTR modulators, I wouldn’t operate because they may be the one thing that’s going to stop us from needing to operate on patients. As they become more and more widespread maybe there’s not even going to be a real role for us to do surgery, which would be fantastic.

Sarah: Mm-Hmm. It’s going to be very interesting to watch that play out. Absolutely, with the modulators and what they may do. I guess there may not really be an answer to this question, but I’m going to ask it anyway. Are there, are there sort of preventative things that people with CF may be able to do around sinus to sort of make things a little bit easier for themselves or to sort of ease any issues or just something they might be able to do to stop things perhaps progressing? Is there any preventative options there for people?

Dr. C. Banks: I mean, that’s a really good question. I think if you think about the heart of the problem, it’s the CFTR dysfunction and trying to improve that is incredibly difficult. We know that saline irrigations, for example, are better than doing nothing. If you do have symptoms, I mean, it moves away the thick mucus and it is low risk and certainly beneficial. And then if you have isotonic saline topical antibiotics added to that, that’s another mechanism. But the problem is that if the sinuses are not operated on, they’re not opened. If they’re not opened, those topical irrigations, we know we’ve done studies on them, very little gets into that sinus. So yeah, it’s an interesting question because if you don’t have symptoms, you don’t need to have anything done. If you do have symptoms, then the best thing is to try and address it. I think get in and see ENT and get a diagnosis would be one proactive thing you can do and come up with a management plan.

Sarah:  Jen, did you have any thoughts around that?

Jen Hauser: I think just knowing that it’s okay to bring it up in clinic, that it is something that the CF team can refer to an ENT specialist so that the appropriate imaging, the appropriate review can be done to then know a better way forward. So even if it’s a symptom that you think might not be relevant, there’s no silly question and there’s no — Yeah, I think just feeling confident that you can talk to your CF care team about that and then and hopefully be then referred on to specialists.

Sam Lefo: So going from that, Jen, so a say a patient comes forward and they have these symptoms and start to be looked at, could you answer what would be the immediate treatment they would start to get in regards to physiotherapy or inhalation therapy options? And then Catherine after that, if you could follow on from her, what would then be the next steps for surgical solutions that it progresses to be worse?

Jen Hauser: So from a physio perspective, I think I go through — I’ll often if I think there’s something happening, I’ll talk straight away to our consultants, see if we can get the referral to an ENT specialist, and then I’ll usually do the SNOT 22, which is one of those PROMs that Catherine was talking about. So a patient reported outcome measure and get the patients very quick and easy to do, but as I said, there’s often a lot of symptoms there that the patient went, “Oh, I didn’t think that was to do with my sinuses, but I definitely experienced that.”

So I will get them to do that and ask them to take it along with them to their specialists. So, we’ve got that baseline. Then I’ll start to talk about that education of the exhalation phase through their nose. So if they’re not doing any airway clearance devices attached to any of their nebs, whether it’s the chromosome, whether it’s their antibiotic or whether it’s the hypertonic saline, is just getting to do the breath out through the nose, won’t do them any harm, but it may give them some benefit.

And then if our consultant prescribes, sometimes they’ll prescribe those sprays like the Feds or the nasinex, and then I just support them in terms of correct technique to make sure that they’re doing opposite hand to opposite nostril head slightly forward. So it’s actually having some ability to get into where we’re wanting it to get into and then just supporting them on their journey with the ENT surgeon. I work quite closely with one of our local ENT surgeons because I think that’s a very good partnership together. And if there is anything we can value-add, certainly will be involved with helping the patient out with a para sinus.

But I will confess we’ve had small numbers that have used that, and that’s mainly because of the burdensome nature of the treatment. But short term we’ve had some really good results with using that. So that would sort of be our role as physio. I think just we spend a lot of time with our patients, so sometimes the questions come up in that situation and helping putting them on the right path and talking to our consultants or whoever else needs to see them.

Dr. C. Banks: Yeah. And jumping in from your surgical options, I think the first thing is just to really consider the goals of the treatment. I want to help the patients feel better. So if they’re symptomatic, I’m going to look at them and see, surgically, what can I do to improve your symptoms? And then my next goal is to eradicate the bacterial and inflammatory burden of the sinuses. Ultimately the main thing I’m trying to do is improve overall quality of life and respiratory health. So we do know that with surgery, by opening up the sinuses, you then allow those topical medications to get to the areas and there is a degree of control that can be achieved by using saline irrigations, by using intranasal corticosteroids perhaps within those saline irrigations and by using occasionally, tobramycin or mucarisin topically as well.

And after we’ve operated on them just to, just to sort of go through, you know, the way I think about it, the next thing I want to do is just to control the symptoms and an adult. So I can do that just by debriding the sinuses and removing the thick mucus that you can’t get out by doing irrigations just in the clinic rooms with a suction device. And in young children, at my hospital we are developing an endo-suite where the kids will have a very quick little sedation, I can suction out their noses. When we think about surgery, the fundamental problem is that mucociliary blanket just not working and there is stasis and really thick mucus and so things can’t move and drain in the way that they need to. So we actually modify it just to overcome an engineering issue so that the cilia do not have to, you know, move the mucus up against gravity.

So we lower the side walls of the maxillary sinuses and essentially universally will do that in everyone because there is so much evidence that that is the right thing to do. If you don’t do that in your cystic fibrosis patients, you will be going back and you will be revising it. So now the mainstay is we do that straight out and the frontal sinuses, irrespective of how they look, I never, ever touch them. That was my teaching on my fellowship over in the United States. It’s just not worth going there. You just leave that frontal sinus alone. Then the patients will come back and you just continue to care for the sinuses until they heal and then they go back to go doing regular irrigations and cleaning as needed. We’ll tailor the antibiotic regime depending on what their culturing at the time, but most of the time rather than have to give things systemically, we can give them topically, which is I think better because you’re exposed to so many systemic antibiotics and you want to leave those. So, topicals are pretty good as well.

Sam Lefo: I’ve certainly learnt more than I ever did about sinuses, so I do feel like I might need more treatment ’cause I do feel like everything you guys have said, it really kind of relates to what I’ve been going through. But just for everyone else listening where could they possibly find more information about their sinuses and what to do about them?

Jen Hauser: Oh, I’d love to give this as an opportunity to advertise cfphysio.com, but unfortunately we don’t have a lot of information on sinuses on our website yet. And that’s why I was so excited and feel very privileged to be invited to be part of this podcast because when we got it up there, it will be a good resource. But we do endeavor to put some more information up there about the sinuses. And so, yeah, I don’t know. Catherine, are you aware of anything CF specific for sinuses?

Dr. C. Banks: Not yet, but we are just with the head and neck ET surgeons. We are trying to roll out a universal kind of consensus. So we’re at a stage where we are going to deliver a questionnaire to all the ENTs surgeons, and I want to get one out for the respiratory guys as well. So we can kind of make a guideline to best know how to manage these patients and then to provide information and a resource for our patients to actually go to patient testimonials, et cetera. So this is a place, you know, and this is a fantastic opportunity, Jen and Sam and Kirby just to get together because like-minded people, I think we can make something of this that is really going to be fundamental to helping our cystic fibrosis patients. So yeah, watch this space.

Jen Hauser: And take home message if anyone’s unsure when they’re next at their clinic, ask their clinic if they can do a SNOT 22 and then at least that will be able to be a way that they can do some screening, sort of see what symptoms they are having and how they’re impacting their life.

Sarah: Catherine, you took the words right out of my mouth. I was just going to say watch this space, it sounds like in terms of more information and resources for people with CF about sinus. But I think that’s a great tip to Jen too. And I think what you said earlier about, there are no silly questions. I think that’s all we have time for today. I think we could probably do a dozen episodes on this topic and perhaps we will. But I just wanted to say thank you to Dr. Catherine Banks, to Jen Hauser and of course to the wonderful Sam Lefo for coming along with being a part of this episode. Thank you all very much.

Jen Hauser: Thanks very much.

Dr. C. Banks: Thank you.

Sam Lefo: Thank you.

Voice over:  Thanks for listening to this episode of the CF Strong Podcast. Make sure you subscribe on your favorite podcast listening platform so you don’t miss the next episode. And if you enjoyed this podcast, we’d really appreciate if you could leave us a review. It helps other people find CF Strong, or share us with your friends. Also, a quick reminder that the views expressed in the CF Strong Podcast may not be reflective of Cystic Fibrosis community Care’s viewpoints. The podcasts are designed to share information and provide insight into the lives of those living with cystic fibrosis around Australia. This podcast was made possible thanks to support provided by the Australian government and was produced by CF Community Care and CF Western Australia. Our theme music is Spark of Inspiration by Shane Ivers from Silverman Sound. Thanks for listening, and we’ll talk to you next time.

This podcast was published in October 2022. If you would like to share your story, please contact us at admin@cfcc.org.au. We’d love to hear from you and so would our listeners.