Prevention and Management of Childhood Bronchiectasis

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Moderator: Emily DeBoer
Speakers: Aparna Rao, Matt Abts, Sarah Hofman-DeYoung

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Yes. We’re going to move right into the next session. If anyone wants to stand up and come down closer so you have a better view, you are welcome to move around. Let me just put this on. So I think I have been waiting for this session for how many years is this conference? 11 years. This is one of the reasons we’re in the meetings and we’re seeing the patients because everyone wants to know, do I need to fix that cleft? Do I need to get the swallow study? Do I need to do this? Do I need to do that to prevent bronchiectasis? And I say, I don’t know yet. And I think this morning in the best of poem, it highlighted, are we doing the right studies? Are we doing too much? Are we doing enough? And those questions that we have ongoing. So I’m very pleased to moderate this session on preventing and treating bronchiectasis here in the aerodigestive setting. And I’m joined by Dr. Aparna Rao, who’s Associate Clinical Professor of Pediatrics at UC San Diego School of Medicine, pulmonologist and medical director at the Center for Pediatric Aerodigestive Disorders and Airway Surgery at Rady Children’s Hospital, who’s going to tell us hopefully how to prevent it. And then if it’s happened, Dr. Matt Apps is Clinical Associate Professor of Pediatrics at University of Washington, pulmonologist, medical director of bronchoscopy services, and the co-director of the aerodigestive program at Seattle Children’s. Okay. Thank you. And he’s going to talk about treatment once it’s happened. Aparna, why don’t you come on up? Yes. The screen is shared. Should be good. And we’ll do this. And then that should take care. That way the thing left is… We’re a very well qualified IT person. Well, I don’t know whether I’ll have the answer, but I have some hypothesis which can be applied. So I think this is a great conference. I’m really enjoying myself. One thing we all share is a common vision that we get all children all over the world feeding, growing, and having the least amount of respiratory infection with the least amount of medication. That is a shared vision between all of us. So the question… We are already doing a lot for the lungs, but how do we prevent development of bronchiectasis? What do we… Let’s see. That’s this one, right? Yeah. You can use this thing too. Okay. So just click on it. Okay. Okay. Okay. So this is how we prevent it. The pediatric aero digestive team all across the world globe can protect the lungs and prevent bronchiectasis. This is our team and thanks to every individual expertise, we are getting better at it. What is… It’s not… Is this the… Okay. Okay. So before… Let’s talk about bronchiectasis first. It is a chronic lung condition which is characterized by productive cough and repeated lung infection and progressive loss of lung function. It is shocking that there are 3 million children all over the world with bronchiectasis compared to the cystic fibrosis population, which is 70,000. Research is increasing in non-CF bronchiectasis, but we have to do more. And as we all know, gold standard for diagnosing bronchiectasis is a high resolution CT scan. So before we talk about development of bronchiectasis, we need to talk about cough. Cough is the most common presenting symptom in a pediatric office. And we think of cough after acute respiratory infection, which is expected cough, and there’s a non-specific cough and a specific cough. This is all a big overlap. And as a combined team, as a collaborative thoughtful team, we need to figure out which category this patient belongs in and then address that cough. So what is normal or expected cough? When a child has a cough or acute respiratory infection, we expect that most children, 50% of the children should improve in 10 days. And by 25 days or 3 weeks, most children are completely free of cough. Children less than 5 years, as we know, have multiple respiratory infections and if they’re in a daycare, good luck. However, any cough that is more than 4 weeks needs our attention and we need to work collaboratively or individually or bring these patients if we think they need more attention to the aero digestive team. So let’s talk about specific cough because that is very pertinent to our aero digestive population. As pulmonologists, otolaryngologists or medical providers, we know the cough that is some child is coughing in the clinic. We know what kind of a cough it is. If it’s a very barky or a brassy cough, we know it could be tracheomalacia. Honking cough is the one which we all dread because that is the one which is very difficult to treat, at least in my opinion. This is one cough that gets me stressed out when I hear that. I’m like, okay, this is one that will need more attention. There is also a specific cough that’s a chronic wet cough that should get attention because if it’s lasting more than 4 weeks, we need to kind of think about it in depth. And I talked about specific cough, but the aero digestive symptoms that we all see in our clinic are very similar. We have a cough, we have a wet breathing, we have children gagging, choking, and then coughing. It could be dry cough, it could be wet cough. Parents talk about rattling chest. They talk about wheezing, which is all presentation in our aero digestive clinic, which is very, very diverse when they come to us. So if you think of children with no associated comorbidities, a child who’s in daycare, who has chronic productive cough, there is entity which most pulmonologists and a lot of us know, which is protracted bacterial bronchitis, very common preschool kids. It has been studied extensively by Chang group in Australia and the bowel has neutrophilia and the most common bugs that are, or bacteria that are isolated are H influences, strep pneumoniae, morexella, and staph aureus. And this is thought to be a precursor to non CF bronchitis, CF bronchiectasis. So when we hear a chronic wet cough as a symptom that has not resolved, it’s protracted bacterial bronchitis. And this is what needs, it’s a description just like dysphasia. Dysphasia needs etiology assessment. Similarly, a wet cough, we may need to do an in-depth analysis. So just now moving on to how long it could be and how it develops. This is the spectrum. This is a spectrum patient can present with chronic wet cough and it can resolve. It can resolve with antibiotics for two weeks. That’s protracted bacterial bronchitis. Sometimes it needs four weeks that is extended. And that could be a recurrence, which is well noted in literature. And if these patients don’t resolve, they move into the spectrum of chronic suppurative lung disease where they may need IV antibiotics. So these are the children whose imaging is also, does not show bronchiectasis, but they do have this chronic unresolved wet cough. And very soon these children can also develop bronchiectasis. The timeline is very controversial. Some people say five years, some say eight years, but we don’t have good literature talking about the trajectory of how you develop non-CF bronchiectasis. So I want to present a few cases that I saw in our aero digestive, which presented with chronic wet cough without swallow dysfunction. So we had a two year old who presented with a chronic wet cough seen in a pediatrician office. First told, oh, it may be a post-viral sequelase. So don’t worry about it. She was then started off with inhaled corticosteroids. Parents are distressed, she’s coughing day and night. They’re going from pediatrician office maybe four or five visits. Then they come to my clinic. Then I hear the symptom. I give her a course of antibiotics, but since I work in the aero digestive group, I have connections. So I made urgent referral to the aero digestive clinic because this was not fitting into the specific non-specific. So it was getting me worried. So, and this was her initial x-ray. So I looked at the x-ray and I was like, there is something wrong with the x-ray. Maybe the right side looks hyperinflated. Maybe there’s something in the right middle zone. You know, x-ray is like a piece of art. We can interpret it in many, many fashion. So I sent her to the aero digestive clinic and this is Dr. Bringer’s bronchoscopy. We all were doing the bronchoscopy together. My video was not as good as his, but as we are going through, we look at the intrathoracic trachea, it looks good. Then we go to the right main stem and guess what we find? There was a foreign body sitting there, which Dr. Bringer and his team extracted very nicely. And guess what? We prevented bronchiectasis. This child never developed bronchiectasis. The cough results is doing really well. And as a combined collaborative, thoughtful team, we could identify a foreign body way earlier and not later in life. So we prevented bronchiectasis. So this is one patient that we all work together. The next child is a teenager who’s a 13 year old presented with a wet cough since this is her first clinic visit and eight years of age. Typical history of getting the theme middle ear infections, sinus infections, treatment for asthma not improved. We saw bilateral ear infusion. We could elicit crackles on a clinical exam. The chest X-ray is right there that shows that the one side is probably down on the left and now retrospectively, I can even see bronchiectasis there, but I knew I was not dealing with asthma. So this child had bronchiectasis and we treated her, managed her, which Matt’s going to talk about very effectively. We could not prevent bronchiectasis, but we did not allow her to have a progression and her diagnosis was primary celery dyskinesia. So this is how we as a team work together. So now we talk about aero digestive hemostasis. This is a very collaborative system, just like our teams. We collaborate very well and we need every system well aligned for successful feeding, breathing, and growth. If anything is compromised, the lungs seize it, but lung is a fantastic organ. It compensates very well with minimal symptoms and everybody knows those symptoms. Although our teams are doing a lot, we are all working towards improving the lung health. So it’s a multidisciplinary thought process that is extremely important when we offer surgical intervention, medical intervention, feeding therapies, all of it is done to prevent the, to protect the lung. So when I was digging into literature on bronchiectasis and aero digestive population, this is just a few of it. Every diagnosis that comes to our clinic has potential to develop bronchiectasis. What we don’t know is the exact timeline. What we don’t know is when do we do the imaging? When do we do the same questions? A lot of unanswered questions, which as a team, we could probably get into and improve, but this is, there are a lot of them. So then I was looking for more risk assessments for how, which are these patients who are, who’s developing bronchiectasis. Again, vast literature with multiple risk factors. The most recent one, which was discussed this morning, that said, which is 2023, that neurological impairment may not contribute. Whereas the one which was prior to that 2012, neurological impairment is a big factor and very early bronchiectasis. So here we are still, we’re still going around in circles, but this is all making a headway towards developing some kind of a, you know, protocol or something to develop, to look for bronchiectasis at the right time, not overdoing it, not underdoing it. Our own center developed this medical complexity score where we kind of gave a point to all the core modalities and here’s the key. There could be airway, anomaly, cardiovascular, gastrointestinal, everybody got a score if there was any of these pathologies. So highest score was seven and the lowest score was zero. And what we found was higher the complexity score, more likely we would not advance them with their oral, oral skills. So so that, that again kind of connects that the more complex you are more likely the lung is seeing it all. So then I looked for the population of neurologically impaired kids. I said, what happens to these children? I was looking for review articles. I was looking for, you know, direction on when do we do this? Because this is a huge population that comes to our clinic where parents want to progress feeds or they want to, you know, establish goals. So I feel like this is a, this is a gold standard for how aero digestive hemostasis is breached at every level. So these are the patients that will also benefit a lot from our team. So if you think about this population, this is the population that has more, oh before that, the neurologist developed a score for this population where they give a score of five, where they have really poor motor development, where they do not have head control or no tonal, no tone at all. And these children have the highest respiratory morbidity. So they kind of gave us a score, but if you think about it, average of these children that come to our clinic, they are neurologically impaired. They often have seizures. So we give them anti-seizure medications. So this causes more bulbar dysfunction. So they drool more. So when they drool more anterior or posterior, it affects the lungs. Then when you come down to the airway, some of them may have other airway pathologies that may be contributing to the aspiration. Then you think of our neighbors, the gastrointestinal tract, they don’t have good motility. They don’t have good esophageal clearance. All of it is impacting the lungs. And these children then later on, unfortunately can also develop scoliosis that can pair the respiratory mechanics. Then they have sleep disorder breathing. Most of these children have obstructive sleep apnea or central apneas. All of it is a breach of every step of aero digestive function and the lung sees it all. And fortunately, like I said, lung is a tremendous organ and we have pulmonologists to take care of the lung and they do better and they try to decrease the respiratory morbidity as much as possible. So in summary, aero digestive population is at very high risk of development of bronchiectasis. And as pulmonologists and as aero digestive team, we should be thinking about it at every time. And the chronic bed cough presentation needs in-depth analysis if it does not respond well to antibiotics. And a collaborative team, a thoughtful collaborative team can make every child feed, grow and breathe better with least amount of respiratory morbidity. And this is how we do it. This is our team from Lady Children’s, all stolen from Dr. Breger. Thank you, Aparna. That was fantastic. If I close this and open this and it’s still sharing. All right. We will take questions at the end. So put anything in the chat or get ready and here you go. Thank you. Thank you, Emily. I will. Dr. Rao, that was amazing. Thank you. And I think I speak for most of us that I would much prefer to take care of a patient where bronchiectasis has been prevented as opposed to taking care of an actual bronchiectasis patient. So I’m here to talk about the management of non CF bronchiectasis. What I am not going to do is talk about CF today. And I hope that’s okay. And my job was easy because in 2021, the European Respiratory Society put together a set of guidelines for how we should be thinking about and how we should be managing non CF bronchiectasis in the pediatric community. I have no disclosures. And briefly, I’m just going to talk about the rationale and goals of care. And then I’ll sort of talk about the acute management and I’ll separate that from chronic management of non CF bronchiectasis. And then hopefully this will kind of be a framework for a little bit of discussion afterwards. Bronchiectasis is important and we should care about bronchiectasis because it is not a rare disease. Dr. Rao mentioned the prevalence. And it’s important to also note that at least there’s some pretty good evidence out there suggesting that the prevalence of bronchiectasis is actually increasing over time. And in the aerodigested population itself, there are some reports that suggest up to 25% of the patients we’re seeing in aerodigestive clinics have documented CF or non CF bronchiectasis on chest CT. We should care because bronchiectasis is generally a pretty neglected lung disorder. I think Emily alluded to that when she introduced us. But there are very few randomized controlled trials and there’s very limited evidence in the pediatric population. So we are constantly extrapolating data from the CF community, from the adult world. When you PubMed bronchiectasis aerodigestive in pediatrics, you get about five hits. So there really is not a lot out there and it’s important for us to recognize that. And bronchiectasis carries with it a really high individual disease burden. So we know that bronchiectasis is associated with decreased quality of life, particularly during exacerbations. It’s associated with lung function decline over time and it takes up a lot of healthcare resources. The chart on the left here shows quality of life indices in patients with bronchiectasis who are at their baseline state of health and then those who are in the midst of an exacerbation. There’s a big difference there. And then the graph on the right shows FEV1% predicted on the Y axis and then quality of life indices on the X axis. So as your lung function comes down, quality of life comes down as well. And that’s very relevant. There are lots of disparities in standards of care as well as outcomes when it comes to non-CF bronchiectasis. This is very well documented. There are lots of varying standards. Up until now or up until these guidelines, there was really no international guidance for non-CF bronchiectasis. We know that in patients who have non-CF bronchiectasis versus those with CF bronchiectasis, there’s disparities in lung function. So those with non-CF bronchiectasis have worse lung function and they have less engagement and touch points with pulmonologists and respiratory therapy providers. And then there are lots of major in-country disparities. This is really well documented in Australia and New Zealand. We see it in the Seattle population because we take care of patients from Alaska. So there’s differences in mortality, lung function, access to care, duration of hospitalizations. And that’s a big thing to point out as well. It’s a profound economic cost. So there was a study a couple of years ago from Australia again that showed each hospitalization cost upwards of about $30,000 Australian dollars. I’m not sure what that means. I’m sure it’s way higher here in the US. I don’t even want to know. But really the one big point I want people to take away from this is that we now know that bronchiectasis is very preventable and that’s incredibly important, but it’s also reversible. And there was plenty of evidence out there in the literature as well as in case reports. There was one study that looked at 22 kids with non-CF bronchiectasis and there was 64% of them had some improvement after appropriate management. And there was a 27% of that population improved. This is a patient of ours from Seattle who had, I think this is a patient with congenital heart disease who had some aspiration issues, developed bronchiectasis. We treated the things that needed to be treated. And three years later there’s significant improvements in the degree of bronchiectasis. This is a patient who got hit hard with H flu and adenovirus, was treated accordingly. And you can see just three months later there’s improvement in that bronchiectasis as well. These are before and after pictures. This is a case report in the literature of a patient with a foreign body. So you see some pretty gnarly bronchiectasis on the left there. And then just six months later after foreign body removal, almost complete resolution of that bronchiectasis. So this is a very treatable and reversible disease. So the guidelines talk about the importance of early treatment in children and their recommendation is very straightforward that whenever it’s possible interventions to prevent or reverse bronchiectasis should be undertaken. The goals are to optimize lung growth, to preserve lung function, to optimize quality of life, minimize exacerbations, prevent complications, and if possible reverse that structural lung injury. And that’s kind of based on like a couple important concepts. One is that adults with bronchiectasis have worse disease and poor prognosis, and that up to 80% of adults with bronchiectasis are symptomatic from childhood. So hitting this hard and hitting this early is incredibly important. And within our aerodigestion community, it’s also really important to mention that we want to treat the disease, not the symptom. I know this is very intuitive and everybody knows this, but of course, if bronchiectasis can be treated, right? But if you take care of the things that are causing that bronchiectasis in the first place, whether it be aspiration or whatever, that’s of utmost importance. And that’s what we do a lot of in aerodigestive. So onto the guidelines. I’m going to kind of rapid fire through all of these and then hopefully again, it’ll be a framework for discussion afterwards. But the ERS process was basically to put together a panel of multidisciplinary experts and families to ask clinically relevant questions, to stratify those questions based on importance and to arrange them into like a PICO versus a narrative format. They did a systematic review for each of those questions and then did an assessment of the confidence and quality and strength of each recommendation. So pretty straightforward approach. And I’ll first talk about acute management. The first question is a pretty, I think controversial one within pulmonary is how do you even define an exacerbation? I think that’s like step one. And it turns out it’s really hard to do. And based on these recommendations, really, they mentioned that all you need is cough, respiratory symptoms for three days or more with or without increased speed and production. This is a conditional recommendation, of course, a low quality of evidence, of course. But what the authors do point out is that we shouldn’t really be relying on changes in chest oscultation or X-ray. Obviously, if there’s positive findings, that’s very helpful, but in many cases, oscultation, chest X-rays are negative. We also can’t really rely on systemic symptoms and blood markers. They’re not very specific. And it’s important to mention that there are certain patient populations that we need to pay a little bit of extra attention to. So those who have severe symptoms like dyspnea or hypoxemia are automatically characterized as having a severe exacerbation should be managed accordingly. Those with a high risk primary disease like an immunodeficiency, we should really have a lower threshold for treating a suspicion of a bronchiectatic exacerbation. For those who have developmental delays who really can’t express themselves or can’t articulate their symptoms, we should probably have a low threshold for treating. So if we do diagnose an exacerbation, how do we treat it in regards to antibiotic choices? And the recommendations are very straightforward. So we recommend a systemic course of appropriate antibiotic for 14 days. This is a very strong recommendation, is really the standard of care at this point. In patients with non-CF bronchiectasis who do not have a history of speed and cultures or BAL cultures, we treat empirically and the antibiotic of choice should be Augmentin or Amox clavulanic. And there’s data behind that, which I’ll show you in a second. However, if the patient has had cultures, obviously we want to treat based on those cultures and sensitivities. And again, this is considered the standard of care. The choice of empiric antibiotic is based on really one single high quality randomized control trial that compared Augmentin to Azithromycin to placebo. And the Augmentin was superior to placebo. The Azithromycin did show some improvement, but it did not meet statistical significance for superiority over the placebo. So if you are treating bronchiectasis and exacerbation empirically, please use Augmentin. And then a quick note on eradication. I think this is a little bit controversial as well. And a lot of this comes from the CF literature. Fortunately, in the pediatric world, Pseudomonas aeruginosa is not very common in non-CF bronchiectasis. We see it sometimes. If we do see it, there is a role for eradication. So if you have a patient with non-CF bronchiectasis who grows Pseudomonas on a BAL culture or speed and culture, we should probably try to eradicate. This is again, a conditional recommendation, pretty low quality of evidence, and there’s no pediatric studies to support it. Adult studies do suggest that eradication can improve quality of life, decrease antibiotic use and decrease exacerbation frequency, and has a pretty lasting effect up to about two years. The guidelines themselves have a really nice algorithm for how to do this. So I don’t have the time to go into the details, but every time I have a patient with non-CF bronchiectasis who grows Pseudomonas, I can refer to these guidelines and they give a pretty well-described way of treating both with systemic and inhaled antibiotics. On to chronic management, should we be using airway clearance therapies in non-CF bronchiectasis? The answer is a resounding yes. This is a strong recommendation, again, low quality of evidence, but it’s important that when we do airway clearance that it’s age and developmentally appropriate and that it should be taught by an experienced chest physiotherapist. So in Seattle, we just got some additional FTE and we now have respiratory therapists at our hip for every clinic and every OR day, which has been fantastic. So it’s improved my workflow. Our patients are happier. They’re getting the things they need in a timely fashion. Our therapists can schedule zoom visits. They can see patients in person for vest fittings and coffices trials and things like that. So it’s really important that we target airway clearance therapy to the patient’s needs, to the family’s needs. And I think that’s probably the most important part of this recommendation. I think we all agree that airway clearance can be really helpful. The limited evidence out there suggests that it can improve lung function, quality of life, as well as freedom volume. And the guidelines also have a nice graphic where they kind of talk about all the different types of airway clearance on the Y-axis. On the X-axis is sort of like patient age, so infant to adolescent, and which of those techniques or which of those modalities are best for what age group. So that’s really helpful. Should we use mucoactive agents? I’ve stratified this into two categories. One is sort of mucolytics like Dornase. And the answer to that is absolutely not. There’s no evidence that it helps in non CF bronchiectasis. This is a strong recommendation. And as other talks have noted, it can actually increase exacerbation rates and worsen lung function, and it can be associated with worse events. So please don’t use Dornase in your non CF bronchiectasis. For things like hypertonic saline and mannitol, the guidelines say that we should not be using them routinely, that it’s a conditional recommendation. But I will say that there can be some benefit. And I think we’ve all seen some benefit using some of these agents in our patients with non CF bronchiectasis. Some of the scattered evidence out there suggests that it can enhance airway clearance, improve quality of life, improve symptoms and extend time between exacerbations. It should be noted that if we’re going to trial something like hypertonic saline, we should always be doing it with a beta 2 agonist to prevent excessive bronchospasm and airway hyper reactivity. And then what about long term antibiotics? Again, there’s been some great talks on this, so I won’t go into a ton of detail. But should we be using macrolides in kids who have recurrent exacerbations? And the answer to that is probably yes. And there’s some good data backing that up too. This is a strong recommendation. And the guidelines suggest that we consider using macrolides in patients who have had three or more outpatient exacerbations in the last year, or at least one hospitalization in the last year. And the evidence suggests that it can decrease exacerbations by about 50%. It is important to know, like we’ve heard in prior talks, that it can increase the resistance profiles and it can have a lot of drug-drug interactions. It can affect QT prolongation. And you tend to have to need a fair amount of adherence to see its maximum effect. This is just a graphic from one of the only, really the only pediatric studies looking at azithromycin versus placebo. The Y axis shows the proportion of children who are exacerbation free. So over time, the azithromycin group, which is in blue, goes longer without a subsequent exacerbation while on azithromycin. Should we be using asthma therapies, things like beta-2 agonists, inhaled corticosteroids in patients with non-CF bronchiectasis? And the answer to that is probably not, unless there’s some overlap. So of course, patients who have non-CF bronchiectasis can also have airway hyperactivity and asthma. So in those patients who have TH2 type inflammation, who have documented airway hyperreactivity that’s reversible, we should be using these medications. But if it’s a straightforward patient with non-CF bronchiectasis, we really shouldn’t be using inhaled steroids. There’s really no role for beta-2 agonists either. And there’s a lot of potential side effects. I think Dr. Besch gave a great talk yesterday about some of the potential consequences of inhaled corticosteroids. And I think we under appreciate that. And I think I probably use inhaled steroids more than I should as a clinician. And so this is something I need to kind of think about and probably change my practice on. And then the guidelines go on to talk about a lot of sort of just kind of basic recommendations. So of course, nutrition should be optimized. We should encourage exercise, which is in and of itself a form of airway clearance. Patients should be immunized. It’s important to have psychological and educational support. So when you think about your aero-digestive clinics, do you have social work available? Do you have psychological support available? I think probably most of us don’t. It’s hard to come by, but it’s really important when we think about holistic care for these patients. And then they also talk about surveillance practices, which is a little bit out of the scope of this talk. But they do make recommendations about how often we should be seeing these patients, how often we should be getting sample collections of sputum, doing lung function testing, and then repeating chest CTs and things like that. And that’s really it. So I’ll just leave you with one thought. I’ve taken care of two almost identical patients in the last month or so, one of which lives in Seattle and Laurelhurst. I don’t know if you guys have ever been to Seattle Children’s Hospital, but it’s sort of like right in the middle of one of the most affluent neighborhoods in all of the country. And you could walk to the hospital from this picture that we’re looking at on the left. And it just kind of brings to light the contrast and the disparities that we see in our clinics. And then you have Point Lay, Alaska, which is like up in far northwest Alaska, where patients have to get on a sled and then get on another sled, and then three prop planes, and then a commercial flight just to get to Seattle for their air digestive visit. And how you would think about, assuming both of these patients have bronchiectasis, how you would think about their management. Like, what do you send our friend from Laurelhurst home with versus our friend from Point Lay? And I think that kind of helps highlight some of the details and idiosyncrasies about how we should be thinking about bronchiectasis and management. But anyway, thank you guys very much for letting me talk. Maybe we’ll have conversation. Well, that was just a fantastic ending to keep our questions coming. Oh, please start us off, Paul. So thank you, Esparitax. That’s great. I care more about bronchiectasis. It’s important thing to me too. So the guidelines are great, except I cannot, I have a hard time with the three days. Like, and then you’re going to say they need to be on, not you personally, but they need to be on azithromycin if you have more than three exacerbations a year. Everybody’s going to have more than three exacerbations a year with that definition of exacerbation. Like if 50% of normal health that gives a non-specific cough after a viral infection will still have cough. Why does a kid with mild bronchiectasis cough for three days merit 14 days plus potentially of antibiotics? That part is, I use 10 days because I figured that’s where it’s like 50/50. And if I don’t, wet cough and fatigue, that’s what it usually is, right? And not usually, they’re not often sicker than that or, you know, it’s just that and hard enough to get pediatricians who are of course trained to not over utilize antibiotics to treat in that setting. And I tell my patients, if your pediatrician says no, just call and we’ll give them for those criteria, you know. But three days, is that, you guys use three days as their threshold? Not always. And I think just to go back to my last slide here, right? Like, you know, you think about how we approach that. You have a patient from far away with limited resources who’s calling in and doesn’t have a lot of options, have three days of symptoms, they have known bronchiectasis. You know, there’s a lot of nuances to this, right? Like what’s their history? What have you gone through with this patient? What have you done in the past? But there are certain instances like that where I would have a really low threshold for treating or a patient with an immunodeficiency or something like that. But I agree. I think it’s a pretty soft guideline. It does come, most of this stuff comes from Australian literature, where there is a lot of disparity and a lot of like distance from, you know, hospital centers to where patients are living and stuff. So I do think that plays into it for sure. But yeah, if it’s a patient who’s like a couple blocks away, you know, who has good follow up, a good pediatrician, I wouldn’t necessarily go ahead and treat that with, you know, 14 days. Yeah, I appreciate that. And the distribution of severity is skewed where there’s a lot of mild bronchiectasis and the moderate severe is, you know, relative, thankfully, relatively small proportion. And so you’re going to, like we can apply this narrow threshold to a large population have pretty mild disease, you know, and are probably not, you know, because we don’t know what the impact of, you know, this, what may be, you know, how much we’re even preventing, we don’t really know the necessity of it. It’s pretty loose. And we know it’s a, in terms of recommendation, I don’t know. I think it all depends upon, you know, I think this is where we have to use our best clinical judgment. There are recommendations and best clinical judgment, and they all have to intersect for the patient. So the way I look at it, there are good recommendations because maybe, you know, there are times where you could use a recommendation, but the patients we serve, it’s unlikely because they have so many comorbidities. So, yeah. And I think the, maybe, you know, the part of what I’m thinking is, okay, where’s the clinical trial? What do we do? How do we prevent? How do we treat? And I think like my pragmatic approach is if the parent calls and says, this is the cough, this is it, it’s back. It doesn’t matter how long it was. They know if, and then asks the question, well, have you treated them before? Did it work before? Does antibiotics always work for them? They get a cold and they have increased symptoms and they spontaneously resolve back to baseline with no intervention. Like that happens all the time, you know, and I think that I, I don’t know that I’m, you’re, Branka is progressing in those situations. Am I risking that? Yeah. I mean, we see that all the time, right? Like I’ve started to get in the habit of like, anytime I see sort of excessive secretions, like mucopyrone secretions on a bronchoscopy, for example, I’ll send a reflexive viral panel just to know, you know, like, and I think I’ve gotten, I’ve honed in like a pretty high degree of suspicion and I tend to be right a lot. And, you know, like, yeah, it’s just an incidental, like we didn’t know about it beforehand. It’s there, kids better three days later, you know, if I hadn’t gotten that viral panel, what I’ve treated with a brief course of antibiotics for, you know, if the neutral count was high enough, like probably, but knowing that there’s a virus in there, that kind of gives me pause. Yeah. Yeah. Yeah. Hey, Paul, can you take that mic up there? Hi, one of my, my question is, I think that when we are talking about exacerbation, we have to see the patient, we have to check the patient is, is, is the first thing we think we cannot make a phone call to see if he has exacerbation, to define exacerbation. And probably five days is not enough or three days is not enough. I think when the experience is growing in bronchiectasis and now probably they are going to change in, you know, in Australia the, the, the days that we are thinking about. Probably if the patient has crackles, that’s very important sign, clinical sign that’s another exacerbation is going. Because one of the things that we know that defines that we are going in a good treatment is when the patient doesn’t have crackles. If the patient has crackles, probably he’s starting a new exacerbation and that will be, that will be the clinical and defined. And the other question is what is the longer time to use acetromycin in these patients with exacerbations? It’s one month, two months, three months, because I know Annie Chang is, is trying to make a work with nine months of acetromycin and it’s going to be difficult. I am in the, I am in the group that will be studying these patients. It will be very difficult to tell the parents to give nine months of acetromycin with the complications, gastric complications that we could have. Yeah, these are all good points. I think if we, if the parent can make, make it to the clinic visit, absolutely. We would like to examine the patient, elicit the signs, you know, use our best clinical judgment and then decide about further treatment. So that point is well taken. As for acetromycin, I use it in my clinical practice. I use it, I talk to the parent, educate them why I’m using it. And it also depends upon the age group, right? If children are getting multiple respiratory tract infections per year, then I have seen lot of benefit of using acetromycin for a prolonged time. Older kids who get less respiratory tract infection, less exacerbation of protracted bacterial bronchitis. We always have a discussion of how long, and that is connected to the number of times we use antibiotics, number of times we thought about, you know, even repeating imaging to see where, what is the trajectory of bronchiectasis. So there are multiple variables that are discussed with the family before we make a decision of long term use of antibiotics of acetromycin. And two, is it a good time to kind of try to see if we can get off the macrolides? That goes back to Mike. My other point is that, because they are, it is going to be long courses typically when you start because you are instituting maintenance therapy, right? That’s what it is, right? And so you’re not treating a disease, you’re, you’re in a maintenance therapy. So it’s going to be essentially ongoing until you think something has changed, that it’s worth the trial off to see if exacerbations tick back up again. Or if you’ve gotten control of aspiration, meantime, we’re gonna throw whatever, whatever, or the kids just frankly older and they’re not a toddler anymore, and maybe they’re in fewer exposures. But because of that, the having a very tight, tight threshold for what conscious masturbation and that number is going to drive whether you start these long courses, I think there’s real responsibility in addressing that point because the out the consequence means lots and lots of kids living on azithromycin for long periods of time because the threshold is set so sensitively in a without qualification, you know, in the recommendation just in general, because we qualified it right, but when we talk about what we really do, we’ve talked about all the things that qualify for us. But I think it’s a thermison in particular, you know, I think we’ve all seen evidence that can be really, it can be really helpful. I try to limit it to like six to nine months when I can and I oftentimes will do it seasonally. We’re over the winter months or the viral season like we will use as a thermison and then off during the summer where things are a little bit less chaotic. And there’s no evidence behind that. I just think for our patients who tend to get sicker during certain times a year can be more helpful when done that way. But it’s oftentimes also one of the easiest medications to get right and to take so it’s harder to get a vest, you know, home with a patient, it’s harder to get a patient home with some sort of inhaled regimen that they’re taking every day. You know, it’s a lot easier to tell a family pick up, you know, 90 days supply was it there my son take it every Monday, Wednesday, Friday. And I think for some of our patients like that can have a huge impact. pharmacy that makes time every 10 days for a liquid preparation, because they won’t, you know, allow them to suspend, you know, constant reconstituted at home, which is a super pain. So in thinking about a crush tablets or something, that’s great. But that’s sometimes a real issue. I will close with the comment that echoes what you said that we are not treating a rare disease and yet most of our colleagues are at a conference that has been incredibly well funded and has a very large number of randomized controlled trials to look at all these studies for a very small population of children around the globe. And we have to be the people to change care, like Aparna started. And we have to do the studies because we have to make sure that three days is the cutoff and CF because you get worse lung function if you don’t give them antibiotics. And we don’t know those answers of if when we treat all these kids, if the negatives of all the antibiotics is worse than the positives of the antibiotics on lung function. Work to do. Thank you.

The daughter of Sanjiv is Anika

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