I think we'll just go ahead and start with introductions here. So welcome. This symposium is, the title is Key Clinical Steps in Transitioning Hypercapnic Patients to Home Mechanical Ventilation. So we'll go over how to transition a patient with chronic respiratory failure who is admitted to the hospital where there's ICU on the floor, and how to transition these patients back to the home setting. Specifically, we'll go over the different devices so that we can set the stage. And then we'll talk about the obesity hyperventilation, the COPD patients, and the neuromuscular patients. So I'm Dr. Michelle Cao. I am a professor of medicine at Stanford University, and I direct the Home Mechanical Ventilation Program. And I'm really excited to work with my colleagues and close friends here who are experts in home ventilation. So I'll first start out with my talk. My talk is not just set the stage so that we're on the same page from positive pressure therapy to a home ventilator. And this is an interactive session, so maybe I'll just take a photo of that so that we can have a lively discussion. How many of you here actually see patients with chronic respiratory failure and manage home mechanical ventilation? Oh, wow. OK. Great. OK. So my lesson objectives are to understand the differences between a respiratory assist device versus a home ventilator, know the clinical indications for using a RAD device versus a home ventilator, know the insurance coverage determination between the two devices, and then the key steps in bringing that patient from ICU to home. OK. So we'll go over the first case. This is a 55-year-old man who presented to the emergency room for shortness of breath. In the emergency room, the patient was noted to have decreased mentation and diaphoresis. Blood pressure is 155 over 133, heart rate 85, respiration 26, and SpO2 of 74 on room air. His ABG, as you can see, 7.18, CO2 is 99, his bicarb is 42, and his BMI is 45. A bi-level PEP was initiated in the ER. While in the ER, code blue was called for respiratory arrest, and the patient was intubated and placed on mechanical ventilation and transferred to the ICU. The repeat ABG, as you can see here, is 7.24, CO2 of 77. Other labs included a BMP of 1,293, bicarb of 32, and negative troponins. Checks X-ray was unremarkable. The medical history is notable for two prior admissions for acute respiratory failure requiring intubation. The patient was eventually weaned off invasive mechanical ventilator support and to nocturnal non-invasive ventilation. What is the next best step? So number one is arrange for a respiratory assist device for discharge. B is arrange for a home mechanical ventilator for discharge. C is referral to pulmonary-slash-sleep medicine for workup of sleep-soda breathing and optimization of PEP therapy. D is A and C, and E is B and C. I'm still seeing people voting. Okay. So not sure that's the correct answer, but my correct answer is E, B, and C. Okay. So arrange for a home mechanical ventilator and also referral to pulmonary-sleep medicine for optimization of PEP therapy. All right. So that's number, case one. So case two is a 75-year-old man who presented to the emergency room for shortness of breath and fatigue. He was admitted to the ICU for COPD exacerbation. He was initiated on NIV with oxygen supplementation, antibiotics, and nebulizer therapy as well as steroids. This is his third admission in two months. He was recently admitted again two weeks prior for sepsis and acute on chronic hypercapnic respiratory failure and required intubation and eventually extubated to baseline oxygen and discharged home to complete his full course of antibiotics. On this admission, the ABG showed a 7.37, CO2 of 76, and on four liters of oxygen. The CT chest showed severe emphysema, mucus plugging, multiple areas of nodules, consolidation, suspicious for aspiration pneumonia. The medical history includes COPD, GOLD4E, coronary disease, AFib, and heart failure. And he uses two to three liters of oxygen, and he's on chronic prednisone. During hospitalization, the patient was weaned again to nocturnal NIV and then eventually back to his three liters of oxygen at home. What is the next best step? A, arrange for a respiratory assist device for discharge. B, arrange for a home mechanical ventilator for discharge. C, referral to the sleep lab for titration and optimization of positive airway pressure therapy. D, A and C, and E is B and C. So the correct answer is B, arranged for a home at chemical ventilator for discharge. Okay. All right. Last case. 37-year-old woman with congenital myopathy who was referred to the assisted ventilation program for urgent initiation of noninvasive ventilation. The patient was vacationing in Tahoe and noted that her SpO2 was less than 80% with symptoms of shortness of breath and palpitations. She left Tahoe immediately, presented to the ER locally near home, and was found to have hypercapnia and was told by the ER physician to see pulmonology urgently. At presentation to the clinic, her FVC is 25% predicted with a normal ratio. Her venous blood gas shows a pH of 7.30 and a CO2 of 81. SpO2 is 86% with a heart rate of 127. She reported that for the past week she couldn't sleep because she was just really short of breath and couldn't be supine, and she had frequent arousals, and she also had a lot of anxiety from this. She also complained of headaches. Because of her symptoms and the VBG, she was sent to the emergency room from the clinic and was admitted to ICU for noninvasive ventilation initiation and titration. She tolerated NIV very well with normalization of the PaCO2. She is on the Trilogy 202 in the hospital with pressure control AVAPS mode with a tidal volume of 450, EPAP of 5, IPAP min and max of 12 and 20, backup rate of 12, inspiratory time of 1, rise time of 3, and an AVAPS rate of 3, and a trigger sensitivity is 1 liter per minute. What is the next best step? Okay, A is a range for a respiratory assist device with a backup rate, which is an ST mode for discharge. B is a range for a respiratory assist device on VAPS modality for discharge. C is a range for a home at chemical ventilator on VAPS modality for discharge. D is referred to the sleep lab for titration and consideration of ST or VAPS modality, and then E is none of the above. Sorry. The answer is C, a range for a home at chemical ventilator with VAPS modality. Okay, so this is a busy slide, but these are the key clinical steps in transitioning a patient to home. So you need to know your devices really well, the RAD versus the home at chemical ventilator. You need to know the device that you need for that specific disease and understand the modalities and the indications for the disease state. Know your insurance local coverage determination for discharging a patient home, and obviously, you know, what you practice changes this. Use a local durable medical equipment company that has experience with a home ventilator management and a good relationship with the outpatient pulmonologist. And, of course, identify the accepting pulmonologist. Set up remote patient monitoring. Follow the plan. Follow a plan for the patient. And then, ideally, set up this device while inpatient in ICU, and the patient should be stable on the device overnight before discharge the next day. So when I think about positive airway pressure device, this is sort of an umbrella that I look at. There's the AutoPAP category, the noninvasive ventilation category, and then the ASVs in its own category. And under each category, there's more advanced devices. We're going to discuss the respiratory assist devices, the RAD, and the home ventilators. So these are the NIV options, at least in the United States. So I like to break it down into two different categories, the sleep hypoventilation, and then the chronic respiratory failure. So under sleep hypoventilation, these are the RAD devices. The coding is that E0470 and E471 that we all hear about from the DME companies. These are your bi-level PAP devices, and they can do spontaneous mode, ST mode, pressure control mode, and then the VAPS modality is an accessory mode that you tag on and you turn on under the primary mode. They can do low-flow oxygen. It's noninvasive only, nocturnal use primarily, and it does require electricity. As of now, in the United States, you can see that the main one is going to be your ResMed device. The chronic respiratory failure are the home ventilators. These are pressure or volume cycle modes. It also has that specific daytime SIF ventilation mode that's only on ventilators. It can do invasive, noninvasive. It can do high-flow oxygen, and it is portable. It does not require electricity, and there's a number of ventilators out there in the market right now. So the key features between the RAD versus a home ventilator. So the RAD devices, again, remember these modalities. It can only do one mode at a time. It is less expensive. It's a rent-to-own insurance policy. It's a smaller footprint, right? It's much smaller. It's about two pounds. Has an integrated humidifier with it. There's less alarms on it, and it offers remote monitoring with adjustments of the settings remotely. The home ventilators, on the other hand, the noninvasive modes are the same modes as the RAD devices. The invasive modes, as you recognize, are similar to the ICU ventilators. It has an auto-EPAP option, the auto titrating EPAP, AE. It also has the SIF ventilation mode. It can program multiple modes. So it can have, like, night mode, day mode, exercise mode, eating mode, speaking mode, and so forth. There's a, there's a, the cap on the instantary pressure is much higher. It doesn't require electricity, and it can be used nocturnally or full-time, 24-7, many safety alarms. Remote monitoring is available, but you cannot adjust the settings remotely. And then it has DME respiratory support. So the NIV coverage determination for inpatient to home, as you know, currently in the United States reimbursement coverage, the RAD devices is almost impossible to obtain from when you're an admitting, when you're a patient, when the patient is going from inpatient to home. However, for the home mechanical ventilator, you, you know, the, these are the criteria. You just have to have a diagnosis of acute or chronic respiratory failure, neuromuscular disease, hypercapnic COPD, any other hyperventilation disorders. You document that the patient requires ongoing non-invasive support, but is otherwise stable for discharge. And the patient and the required modes are, it would be a stronger case if the required modes are not available on the RAD device. And then discharge is unsafe without NIV continuation, leading to high risk of admissions, ER visits, and potentially death. This is the outpatient, just, just so you know the differences. The outpatient, this is outpatient coverage determination for a home ventilator. And you can see that CMS doesn't have testing involved. It really is just your, it's basically your determination of how critically ill the patient is and why do you need a ventilator over a RAD device, right? So it, you determine that based on your clinical, your clinical notes and how you document. And, and so it's, it's, it's much more easy, unfortunately, in some ways, economically, but much more easy to get the home ventilator. Whereas for outpatient RAD qualification, and you can see here, I just want to summarize this, this slide here, is that you need a lot of testing to get the RAD device for the patient out testing in the out, in the outpatient world. And you can see here in neuromuscular disease, you have to use your PFTs. For the hypocapnic COPD, you have to go through your ABGs, your oximetry testing. You have to document the patient doesn't have sleep apnea. And then for the obesity hypermolation, you also need the ABG, you do need the PSG or the home study to show that the HI is less than five. So it's very difficult to get the devices right off the bat without going through these testing. But this is all outpatient. In the inpatient setting, this is not a criteria, okay? Just so you know the differences, because I, there's a lot of confusion with that where the physicians don't know and they use the outpatient criteria for inpatient discharge. So I just wanted to, to verify that or to let you see that. So know the devices needed for a specific disease, right? So for neuromuscular patients, these patients have progressive respiratory failure. So I try to go for the home ventilator. For hypocapnic COPD, you're using it primarily nocturnally and you're using that high-intensity pressure support ventilation. So the RAD device is, with the backup rate, is sufficient. For obesity hypermolation, you want to treat the OSA component for most patients. So you can use obviously CPAP or the RAD device without a backup rate. And then for, so although patients may be discharged with a home ventilator, you have the option to actually switch the patient to a RAD device for economical reasons. When setting up home vent NIV, know the appropriate mode for a specific disease, right? So just know you know that there are unique modes on the ventilator that are utilized to strengthen the approval process for discharge, such as the VAPS-AE. The AE mode is only on ventilators. And then the daytime sit ventilation is only on ventilators. However, these nodes should not be used universally for every single patient because it depends, because you want to know what you're treating. VAPS-AE may not be ideal for like a neuromuscular patient. So for the neuromuscular respiratory failure, because you're using full-time support, I usually try to go for like a pressure control mode and then the sit mode for daytime, daytime support. For hypercapnic COPD, you want to augment ventilation. So a pressure support mode with a backup rate. The AE mode might be beneficial if you have auto peeping. And then for the obesity-affected ventilation, again, you're augmenting ventilation but also splinting the upper airway. So a pressure support mode with the AE is beneficial because you want to treat the upper airway components. And this is my last slide just to go over the process. Identify your DME provider with expertise in home ventilation. Communicate with the DME provider on discharge. Set up in the ICU. Get your case manager involved so that the RT from the DME company can come in the ICU, set up the ventilator, have the patient use it overnight to make sure they can tolerate it, they're stable before discharge. And then arrange for the outpatient pulmonologist, accepting pulmonologist, and then you can discharge the patient safely after that. Thank you. Our next speaker is Dr. Lisa Wolf, and she'll be discussing obesity hyperventilation syndrome. Okay. So I wanted to take a little bit different tack on obesity hyperventilation today just because we've seen so much of the standards from the American Thoracic Society. That being said, if I have any trainees here today and you're looking at the one paper to look at for obesity hypervent before you go into boards, it is the ATS guideline. So just know that going in. The first thing I want to talk about is the epidemiology, which is super interesting. We know that the BMI greater than 40 is going to be about 8% of the population, and we know that that percentage rate is increasing. On the epidemiology, it really is 50-50 men to women. The reason that women look like they're a higher percentage in this group is because they get older, right? So you'll see that cardiovascular disease is the highest source of mortality in this group, and the men die younger. But if you really look at the numbers, it's 50-50. But here's the kicker. Only 10% of individuals with severe obesity and OSA actually have CO2 retention during the daytime. That means that this is a super small percentage of the population, 0.4%. So we're essentially talking about an orphan condition here where we have to ask ourselves a question. Why is it that some people with obesity and severe apnea will daytime retain CO2, have an inability to do appropriate ventilation, and most people will not have that problem, even association with obesity? Now, one reason why it looks like the amount of obesity hypoventilation is bigger than it is, is that 75% of people with obesity hypoventilation actually have concurrent COPD. And it is likely that their CO2 retention is related to their COPD. The biggest struggle that we have right now in terms of the epidemiology and treatment here is that all the European studies are COPD super skinny guys. And in the U.S., the vast majority of our patients with COPD are big, heavy guys. And so separating out what do we do for OSA with obesity hypovent, what do we do with COPD is really challenging. And you're going to see that the reason that this is so challenging is the role of oxygen. And we'll get that in a minute. We all know the physiology. We all know that by the time you get to a BMI of 40, your FRC is reduced and your ERV is down. And that is going to significantly contribute to what's going on. But it's certainly not the whole picture. We also know that because of obesity's impact on the pharynx from here to here, we see narrowing of everything from the upper airway, narrowing of peripheral airways because of chest wall, elevation of the diaphragm, a lot of that because of hepatic steatosis, raising up that diaphragm, increased air trapping, big issues with VQ and mismatch because we get atelectasis. And we've got to overcome that atelectasis. And then, of course, sustained hypoxemia. The other thing we're going to see, and hopefully you can see it better than me, but is this area to the very end of the slide where we show that sleep disordered breathing and issues with elevated bicarb seem to be driving a lot of this problem. The next thing we know is that if we look at these guys, their biggest issue is a high mortality very easily seen, starting at about three months after discharge. Very few disorders can we say in the pulmonary world that if we wait more than three months, we're going to see significant elevation and mortality, not just readmission. And the biggest factor with that mortality is cardiovascular disease. It is not issues with the lung itself. However, what is the source of cardiovascular disease? Pulmonary hypertension and HFPAF. Yes, there's also arterial disease that we need to look at, but these are the two that we need to address the most aggressively. And what's super interesting is that there's a pro side and a con side to this. We know that obesity hypoventilation drives heart failure, coronary disease, pulmonary hypertension, as I just said. But you know what? The ischemic preconditioning that occurs due to the intermittent hypoxemia at night actually may be a little bit protective. And so it's interesting how we need to balance these two things. But it is going to make a difference here in a minute. What we know is that if we can successfully treat with Pap therapy, we see a change in the following two biomarkers. So the biggest difference between maybe a time that you heard me speak last year and this year on this subject is the question of biomarkers. We're going to need to change how we follow these patients. Two important biomarkers that have been published over the 12 months, the last 12 months. The first is the use of cardiopulmonary coupling, which is a way of analyzing the RR intervals that occur overnight as we're doing polysomnography. What I'm telling you is that, how many folks here are asleep? Oh, excellent. So what happens is, you know, we've made the case that all home testing is probably inappropriate for obesity hypoventilation. And we've made that case based on CO2. And I'm not saying CO2 isn't an important biomarker because it is. But I think that the case that we're going to be making going forward is that full EKG as part of full polysomnography is going to allow us to look at this elevation in low frequency coupling and a narrow banding in the RR intervals throughout the night. We know that if our NIV improves cardiovascular coupling, then our mortality goes down. And how do we know it? Second new important marker to think about. High sensitivity troponin. Now, we don't think about this, right? Troponin's a cardiac thing. It's not a pulmonary thing. But the reality is, we know that the value of NIV is the importance of our cardiovascular markers. So I think that when we look at what's going to be coming down the road, it's going to be cardiopulmonary coupling analysis in the sleep lab and watching what's happening to the H-troponin levels as we start NIV. Now, why is this so important? Well, part of this is this issue of weight loss and how we deal with total weight in these patients. Here's the irony, a little secret that we like to kind of slide under the bed and not tell our patients. CPAP makes you gain weight, right? We all know this, right? Why does it make us gain weight? It's unclear. We don't have a good answer. But what's super interesting is that if you have obesity hypoventilation and you have CO2 elevation, again, CO2 still being a biomarker. If we treat you with either CPAP or NIV, guess what? You lose weight, which means being aggressive about looking at this treatment is going to be important after discharge. The next thing is in our bariatric surgery patients, we know that good outcomes for sleep disordered breathing get coupled with good outcomes in mortality regardless of how much weight they have. And what are some of the other potential interventions? Well, in the olden days, we used to say progestins. But progestins really are not as helpful even if they can address PCRIT a little bit. They're so associated with DVTs and worsening pulmonary hypertension, we don't want to use them. So what drugs should we be looking at? Well, the first thing is that there was a large RCT that was done this year looking at Diamox. And what we showed with Diamox is that it basically is even. So here's the result of that study right here. Whoops. Here it is. That little pink box in the middle where you see the black band there. If it was a big difference, we'd see this going to the acetazolamide side, which is the left side. And we just see it stay right in the middle. And so although acetazolamide may be helpful in seeing that your CO2 goes down, it's really not giving us the mortality benefits that we're looking for. So what are some of our other options in terms of getting rid of the fluid that is really worsening sleep disordered breathing as our obesity hypoventilation patients lay down? Well, it is cardiac, right? So we call this the wine bottle hypothesis. Well, I call it wine bottle because it sounds more fun. But the real cardiovascular people say that it is fluid, rostral fluid shifts. And so in other words, we're going to say as you're upright, you collect fluid in your legs all day. And as you lay down, those fluids are going to drive up to both the pharynx here as well as increasing central chest pressures. And if we can prevent that fluid from being there in the first place without medications like Diamox, we're going to do better. The best way to do that, and if you can see that up here in my upper corner, is to get OTs, so occupational therapists that are boarded in what we call lymphedema therapy. We think that should be started while they're an inpatient, continued through to the time they're an outpatient. It gives us prevention of those fluid shifts, and it helps to treat that hef-pef that they all have without using medications that seem to be problematic. And by the way, one of the other reasons they can be problematic is the more we get a metabolic alkalosis, the more CPAP-induced centrals we're going to see in this population. So get your OT involved when the patient's in the hospital. Next, I want to talk to you about the coolest stuff that has happened since you were here a year ago. And this is the use of weight loss therapies, which will have two values. One is that, right, they get rid of weight. But two is that they fundamentally change ventilation, and they improve the cardiac functions directly. So the first is going to be the use of leptin therapy. Now, leptin therapy is not currently on the market, but there have been two studies that have been very, very promising in the animal models. So what you'll see here with the little mouse over here is that the leptin is injected through the nose. They sniff it up. It goes into the brain, and it changes how the brain handles ventilation. We've said that these obesity hyperventilation patients have a fundamental abnormality that makes them different than other obesity patients, and it's the fact that their leptin resistance is so profound. So looking at the future where we maybe gave them people leptin nose sprays is not too far from you. And the next, which is a little bit closer, is the use of GLP-1 or SGLT-2 drugs. The best data is with the well-established role of the GLP-1s. We are looking at data from the SGLT-2s coming forward. So let's take a look at what those drugs do and how they work. So the first thing is with the leptin. In the leptin, we're going to see that there's a significant improvement in central factors that increase ventilation, and here's the cool part of it. If we look at these patients, they start breathing before they've lost a gram of weight, right? So we're now treating the disease directly. And then if we look at the SGLT-2s, these are the drugs that are developed for diabetes, but what we found is they reduce rate. They reduce the ketogenic effect, so improving CO2 directly. They have a reduction in sympathetic activity, which means that they have better control of ventilation. So you want to look for these drugs first as a primary way of impacting, and in the data that has been already produced, same thing. The AHI reduces from severe to moderate before they've even lost a gram of weight. And if we look at the alternative, which is what we're doing now, and I want to see a show of hands of this. If you have a patient who has obesity hypovent, and they have the problems that Dr. Kao was just talking about, where the DME isn't being helpful and the insurance company isn't being helpful. So instead, we send them out on oxygen, say, come back. Yeah, we're seeing a handful of people still doing this. I think it's happening more than we're willing to admit in this room, because if you're in this room, you're a rock star to begin with. But what we know is the following. Oxygen alone increases CO2. Oxygen alone is problematic for hypoventilation, making it worse. And even if you give them NIV, it's not a perfect situation. Because if you give them NIV and they still need oxygen, something else is going on, which either means they have concurrent DVT, PE, and chronic thromboembolic disease for the most part. And because I'm probably completely out of time, am I out of time? I have five minutes, woo-hoo, okay. We are going to talk about the fundamental paper, which I said is the basis for where you guys should be answering questions if you're studying for boards. Okay, all right. This graph comes out of the American Thoracic Society document on how we should be treating obesity hypoventilation. Now this is a big graph, and it's very difficult to get through. So I'm going to change this to what I now have been referring to as the step-up, step-down method for treating obesity hypovent. If you think about it this way, it's going to be a lot easier. First, let's talk about the hospital patient, because that was the topic I was given today. Hospitalized patients need to start with very high-level therapy, with backup rates and the ability to be nimble as fluid shifts are going to be prominent in the first three months. So that would be a VapsAE mode that you can get on a home mechanical ventilator that includes a backup rate, and you should only use oxygen if absolutely needed. Over the first three months, those fluid shifts and the cardiovascular issues will be much better treated, and you can now do what we call the step-down mode, which is that after three months from hospitalization, bring them back to the sleep lab, start at CPAP, start on room air, and see if we can get them down to a small, easily supported amount of CPAP. Now that step-down method I just described is for patients that present in the hospital and the ICU. Now let's talk about the other side of this slide, that is the step-up method. Step-up is for patients that present to your clinic as an outpatient. You don't want to miss the fact that they have hypercapnia, but the way that you want to treat them is getting them to the lab as soon as possible to start CPAP, straight CPAP therapy. We know that for most of these patients, they will not need oxygen. They will have an ability to resolve their hypercapnia in the first three months, but not necessarily in the sleep lab when you first see them. So if they have a little unresolved CO2 at the beginning, it's okay. Pressure intolerance may be an issue, and if it is, you can go to bi-level spontaneous to improve compliance. And remember that these CPAP devices are pressure limited, so we want to see if we can treat them at the lowest pressures possible. So using things like TI extension, super important. And remember that that TI extension will help to recruit the bases, get rid of the atelectasis, and give you good therapy over time so that you don't have to step up unless you need it. All right. That is the end of my two minutes. So thank you all very much. I mostly want to thank both Dr. Cao and Dr. Salim for putting this session together. And she's going to talk about neuromuscular respiratory failure. Thank you so much. My name's Bethany Lussier. I'm from UT Southwestern in Dallas, where I do neuromuscular pulmonology, neuro-ICU, medical ICU. So my area of interest is actually getting patients from the ICU to home. So hopefully this comes in a little bit helpful. I don't have any disclosures that are particularly relevant to this talk. The objectives today were really, this is supposed to be a super practical approach. I'm not going to throw a bunch of data at you. So I'm going to get towards the middle of the talk. It's going to be mostly case-based and trying to give you guys a practical approach to getting your patient literally out the door. I wanted to spend just a second, just a minute, on the physiology and how it's different from others. It's an extreme balance. And these patients tend to be tenuous, depending on the criticity of their disease. And it varies from pediatrics to adults. The chest wall itself in pediatrics is hypermobile. In adults, it's not. And it impairs the gas exchange with the hypermobility of the chest because of the dynamics of it in a pediatric patient. And by the time they're four, if they're already hypermobile, they lose gas exchange surface. In adults, it's different. They get stiffer. And that stiffness and the loss of elasticity impairs their ability to compensate and increase their tidal volumes. So in a patient who develops neuromuscular weakness and is addressed with a challenge, an added weight to that system, you're going to go load that left side of that lever. When you do that, they have no ability to compensate appropriately. And so they may increase their respiratory rate. In a normal adult, you'll increase your respiratory rate and your tidal volume. But in somebody who has weakness or stiffness of the chest wall, loss of elasticity, you're no longer going to be able to do that. You're not going to be able to increase your tidal volume. What ends up effectively happening is you shorten your TI min. You're shortening your TI time of that curve. And so it's somebody who is already weak. And now you're putting them at a disadvantage because they can't even generate that pressure because they're getting weaker and weaker and weaker. And now you go and you load that other side of the system. And you're addressing them with a challenge. They increase their respiratory rate. They shorten their TI time. And now they're not able to generate that pressure at all. So these patients may be toying with hypercapnia all the time. They're able to compensate. And then you go in a hospital situation. They get increased secretions. They have heart failure. They have even gastroparesis, whatever they end up presenting with, a general pneumonia, even RSV. And they end up coming into the hospital. There's an increased workload. And they weren't able to compensate even at baseline. They were barely getting by. So these patients come in and they're tipped over super easily. So it's different for somebody when you're assessing the patient. It's difficult on the inpatient side. This isn't advancing. Sorry. It's kind of important to figure out where you want a trajectory and being able to identify where the patient's going next. There's a big, it's a big range on which is going to be rapidly progressive or a slowly progressive condition. And you have to figure out where they are in that trajectory and how fast they're getting there. Because it's going to determine how aggressively you're going to start somebody on non-invasive ventilation. It is also going to be important to say when you have your patient on an inpatient, how far were they already when they came in and how close are they to the end at the point that you're seeing them. The goals of ventilation are a little different when you're talking about starting somebody on home ventilation who has neuromuscular weakness. And I can't stress this enough. When you're dealing with somebody who has hypercapnia, if they're already hypercapnic from neuromuscular chronic respiratory failure, you're probably past the point where they should have been started on support. They're probably past that point. Because they should have been started on support when they started showing signs that they were dipping in their FEC or showing you signs of decreased strength. Because they're not going to show you hypercapnia until they've already started hypoventilating at night. When you think about the mechanisms, you go into a deep REM sleep, the only muscles in your body work into the diaphragm and the heart. If the diaphragm isn't working all the way because it's weak, you're not breathing well. And so you end up with hypercapnia. You have hypoventilation at night. And your pulmonary function and your forces are going to tell you that before they start develop hypercapnia, especially nocturnal hypercapnia. Daytime hypercapnia is going to be much later. So when you have your patients, your goals for your nighttime ventilation is always going to be to improve sleep quality and energy. You're going to offload the diaphragm. When you're at the point where you're developing hypercapnia, you know that you're going to improve survival and improve quality of life, yes. Daytime ventilation looks different for these patients. When you see hypercapnia in a patient with neuromuscular failure, the question is, do they need nocturnal ventilation? And how aggressive do we need to be about that, depending where they are in line? And then the second question is, are they hypercapnic? And is it affecting daytime? Is it because they need daytime ventilation? They're already on nocturnal ventilation, but now they need daytime ventilation. And the approach to daytime ventilation is different from nighttime. It should be for most patients, unless they are completely ventilator-dependent. For non-invasive ventilation, you're going to improve function, you're going to improve symptoms, and you're going to reduce the work of breathing, which will then treat the daytime hypercapnia. I'm not going to spend a lot of time on this. We know MPPV works. We know it works for respiratory failure. It improves survival. Even as much as four hours of use of non-invasive ventilation at night will improve survival in ALS. It improves quality of life. And this holds true even for patients. The survival benefit is not there for patients with bulbar weakness, but the quality improvement is. We don't even do randomized trials. We don't even compare them to patients who are not on ventilation, because it's not appropriate to do. We know they need it. We use extrapolation from Duchenne's. The largest trials are typically in the Duchenne's patients and the ALS population. And we extrapolate this to all patients with neuromuscular weakness. It's probably not appropriate, but we do it. Post-polio CMT, we kind of let them hang out to dry. But when you lump them in, if you look at the Duchenne's data, back in the 90s, they looked at it. And if you developed daytime hypercapnia, you're looking at a seven to nine month mortality. That's insane. But they're telling you, this is where I am on my trajectory. I'm almost there. At the point they develop daytime hypercapnia, they've been hypoventilating at night. The mortality is markedly improved in Duchenne's. The life expectancy has markedly improved since the 90s. We have therapies for it. And we are very aggressive with ventilation. So it really does make a difference. And in addition of daytime ventilation can look a lot of ways. Dr. Cowarty went over, when do you need a RAD? When do you need HMB? There's a lot of things that affect it for my patients. And perhaps my population may be a little bit different. Cost is a huge factor for my patients. So if they only need nocturnal ventilation, I push that as far as I can. So while it's difficult from the inpatient side to get a RAD device, I will sometimes get it because my patients really don't, they can't afford it. They can't afford to go straight for the mechanical ventilation. But for the vast majority of patients that you're going to be discharging, these patients have a progressive neuromuscular condition. At some point, the vast majority are going to, and if they're hypercapnic, they're going to need at-home mechanical ventilation. The criteria is there. The only thing I want to highlight is that the PCO2 on these criteria is already hypercapnic. There's a difference that the European Respiratory Society makes the guidelines. The symptoms are enough. You don't even need to qualify for an FEC. They don't care. It's fine. If symptoms are there, it's enough. And that's honestly probably where it should be. I will say what I focus on for these patients is this MIP. Like, it's weaker than minus 60, so greater than minus 60. But the MIP, if you need to get it to make sure that your patient qualifies and stays qualified, lay them down and repeat the MIP. That's the easiest way to do it. Get them qualified, and then so you have them in follow-up, your vent's not going to be in question. Look, their MIP was terrible. Obviously, if they're hypercapnic, their MIP is terrible. Interfaces. I'm going to go through this briefly, but it is important because your patient's probably going to need more than one, especially. These patients tend to have a lot of issues that you may need to think about when you're sending them home. Macroglossia with a lot of these patients, just the laxity in the jaw, whether they're bulbar or not. So consider the fact that you may need to switch up these interfaces after a week or two. Daytime ventilation, you don't need to use the same one at night. But it is going to affect, one, the leak, which tends to be a major problem in procuring ventilation for these patients. You get a lot of leak with the nasal interfaces, obviously. And paying attention to that when you're choosing your settings is going to be important. And then patients who have secretion issues, you may want to consider that also. The question is always, do you wait for the sleep study? That's the biggest thing. You can use a sleep titration study. You don't have to if you are going to do it. That's one way to use a sleep titration study, but it takes a lot of time. And some of us have access to that on the inpatient side. Some of us don't. I tend to go with number two, where I make a best guess based on what they were on before. And if nothing worked, then I try something different. And then the way that I tend to get most of my referrals from inpatient to outpatient is somebody just slapped them on a VAPS mode because it's the easiest thing to do. And it comes with a nice wide range. And then you look at what the range showed, and then you narrow the range. So this tends to be what most people do. All of these are workable. We can work with almost any of these. If you were going to do a sleep study, and this is the part that I'd like to highlight. This is from the Atlas with Krieger and Meyer. But if you look at a sleep study, you're going to look at the work of breathing. And it's highlighted down here at the bottom. You can have a patient who has neuromuscular weakness, and they will look great. SATs are great while they're sleeping on minimal pressures. And their SATs are fine. N-TITLE or the transcutaneous CO2 look great. And they look like they're breathing OK. And they go into a deep sleep, and the activity of the sternum is going wild. The EMG on the sternum is going wild. They're working, working, working. They're not actually resting anything. And then when you actually increase the backup rate, you'll watch their respiratory rate come down. Their TITLE volumes are addressed by an increase in pressure support. And then suddenly, they go into this nice, flat EMG, and their muscles are at rest. And this is how you fix hypercapnia. You rest them. You rest them. This is my patient who's 46-year-old man, has ALS, was on BiPAP, was doing well on BiPAP for six years. He was doing great. I mean, look at these exhaled TITLE volumes. And the exhaled TITLE volumes are what's measured, OK? So I just want to throw that out there. He was doing great. Trigger was nice and low. Many years, he was on lung volume recruitment to improve that elasticity, keep his ERV where it should be. Gets admitted for a pneumonia, doing awful, develops hypercapnia. He's also on diuretics. And it's not severe hypercapnia, but he's looking much worse. And somebody sent him out on a vent. He shows up into my clinic. The question is, BiPAP ST or VAPS as a mode, OK? The data supports either one. There's not one or the other. As long as you're procuring ventilation for your patient, and as long as you're watching it and making adjustments appropriately, you can use whichever one you want. Moral of that story. This is what he was sent out on. I'm going to highlight in this particular case, this patient came in, couldn't trigger, couldn't trigger his breath. He was breathing at probably 22, if you actually put your fingers on his chest wall. But he was only triggering 12 breaths a minute. He couldn't speak, because the back flow is so high on nasal pillows. And I almost admitted him, because he looked like he was going to pass out. I didn't even bother to check labs on this guy. The point is, if you're going to send somebody out, be cautious about the settings you're on. You may choose to go with a VAPS AE mode, because it's easy to do. And you're going to say, well, it covers the range, and it will adjust to what my patient's needs are. But there's caveats to that in someone with neuromuscular respiratory failure. You really need to ventilate your patient, and the ventilator needs to respond to the patient. If your patient is too weak, and the ventilator is not sensitive enough to your patient, it's not going to be able to react appropriately. So this patient was sent out on a very high IPAP range. The other thing I will say is, when you give somebody a volume-assured mode, a targeted mode, and you give them a range that's 20, it's going to start in the middle. So this guy's going to start at 15. If somebody's not used to a pressure of 15, they're going to balk. And then it's going to slowly go up or down based on the feedback, the exhaled volume back to the machine. And it's going to adjust up or down, and it's slow. If the patient's too weak to trigger that machine, or it's got a lot of leak because he's using nasal pillows, it's not ventilating your patient, and it's going to ramp up the IPAP. You can't give a patient with neuromuscular weakness an IPAP range of 4 to 20 and then have them talk. It's just not going to work. So when you see these patients, if you're going to send them out, and this is it, I want you to focus on the area under the curve and making sure you're ventilating your patient. And you use the feedback from the device. So I have this next. This is what I wanted to show here. If you're going to send them out, send them out on settings that make sense. What you're going to do is you're going to pick the targeted minute ventilation you want or the target volume you want. And you ensure an appropriate eye time. Adjust your TI min and max if you need to. Make sure the area under that curve is sufficient. And adjust. You want to make sure they have an appropriate backup rate. You want to drive them to rest. You want it to look like it's sleep. This is for nighttime. This is for ventilatory support. It's not great for daytime. If you're going to send them out on the VAPS mode, it's not great for daytime because they need to augment activity. And you probably want to transition them to something like nasal pillows. But this is just if you want to start somewhere, I would suggest doing something like this. That's why I put it up there. Try to be practical about this. And then if you're going to use an IVAPS mode, something changes a little bit because you need to choose a target volume. That's why I put it up there, suggested modes. Just be very cautious about what you're doing. And then narrow the ranges once you have feedback from the system. If you need daytime ventilation, this is my plug. And this is one of my patients. And I promised her if I used her picture, I'd put it in there with her book. She's a wonderful horror writer. She writes short stories. She's fascinating. She uses mouthpiece ventilation all day long to write her book. It's to augment activity. But it reduces daytime hypercapnic respiratory failure. And it also recruits and improves the elasticity of the lung. It allows them to do breast stacking and lung volume recruitment. So it's a dual effect. And these are just suggested starting settings if you've never done it. It's a pretty effective way to initiate daytime ventilation without tying your patient to the ventilator to the wall. But it does require a home mechanical ventilator because you need that as a secondary mode. And I'll just last note, elasticity matters. But the absolute success of non-invasive ventilation definitely requires the ability to clear your airway. And so none of this will be effective if you send your patient out who can't control their secretions or their airway. So it's absolutely necessary for those neuromuscular patients to take that into consideration. Sorry, I ran over. Thank you. All right. Our last speaker is Dr. Bernardo Salim, speaking on COPD. It's OK. It's OK. There's nothing up against us. All right. Great speakers. Very difficult to follow. So I will try to do my best. My name is Dr. Salim. I come from Mayo Clinic. And I have to tell you that I do live in two worlds because I'm the director of the respiratory care unit. So I do deal with a lot of the patients coming from the ICU. And one of my most difficult and challenging things is to get them home with non-invasive ventilation. How many of you are you doing high-intensity COPD treatments right now? All right. You have to tell me, how are you doing it? Because literally, some of the settings that the data is coming from, again, the DMEs are going to be very difficult to get them approved. So who, when, how? That is my three words that is going to be driving me through this talk. The first case that I have is this 63-year-old with COPD. You see that over anatoximetry. What respiratory change may correlate with the abnormality shown above? Decreasing hypoxemic respiratory response or hypercapnic respiratory response, decreasing diaphragmatic function, or increasing respiratory drive. I have one vote. All right. I'm going to drive you directly into the answers. And let me see if it goes forward. So literally what you see here is going to be your diaphragmatic dysfunction. This is what is happening with your PSG. You're going to be having a lack of respiratory response. Therefore, these patients are going to not be triggering your bilevel. And therefore, you need a backup rate because you do have a hyperventilation in that period of REM in which in the COPD, the diaphragm is the only muscles that is going to be driving the ventilation. Being in a mechanical disadvantage secondary to hyperinflation make these oximetries to be very common. So this is what you see with the PSG. The second question that I have for you that I think that is a little bit more challenging is what is this mechanical or mechanism by which the high intensity bilevel pap therapy works? Does it work by increasing hypercapnic respiratory drive? Increases the inspiratory muscle strength or decrease in it? Or is a diaphragmatic respiratory strength that is increased when you apply high intensity? I hoped I have more than one vote. Come on. It's early in the morning. I can't justify that it's at the end of the session. All right, lovely. Fantastic. Thank you. So the answer is pretty spread. But I wanted to bring you some more excited mechanism or explanation of the mechanism. There's no changes in respiratory muscle strength. You and me, we believe that the bilevels will be putting the muscles to rest. That is true. But the mechanism that has been proven to be the one that is behind the high intensity is, in fact, increasing hypercapnic respiratory response. Interesting. All right, so I promise, who and when? Who? You need to treat with high intensity patients with a stable COPD with chronic respiratory failure. Also, late recovery phase after a COPD exacerbation. Not immediately, not in the hospital, but four weeks after discharge is what you're going to treat. Now, when you're in the hospital, like I live myself, I do receive patients with non-invasive ventilation with COPD exacerbations all the time. I start here. And I know that if I start the bilevel in the hospital based on the rescue trial, this is going to be a negative trial. This is not going to be helping my patients to readmissions from mortality. But if I let them go, stabilize four or six weeks later, and I start this same high intensity intervention, I can prove that the patient can decrease their hospitalization and mortality. So you do have two phenotypes. And if you want to take a photo, this is the photo that I will take. These are the phenotypes that you do want to intervene with high level BiPAP. The chronic stable hypercapnic COPD, and the post-acute exacerbation COPD. Look at their profiles. One is going to be the infrequent exacerbation good function. The other is the frequent exacerbation with acute NIV needs after the discharge. So these are the patients that the phenotypes that has been proven based on data that the intervention of high level will be doing good in the mortality and readmissions. So I have Dr. Gay here that is one of the pioneers of placing patients with COPD on bilevel. And he was very gentle. And at the beginning, he was doing this low intensity, low delta pressure for which it has been proven not to be effective. Unfortunately, we needed to tie it up, put a large pressure support. That is where the high intensity comes, is high pressure support plus backup rate. And that is where these studies are coming that has been showing not only improvement in the quality of life, but also in mortality and readmissions. So the important question is, above all, what is your target? When you start bilevels in these patients, where are you going to be targeting? And we learned that from two studies. The one from Colin from Germany is telling you that the CO2 needs to be targeted with a decrease of 20% or a CO2 less than 48.1 millimeters of mercury. Look at the pressures that they are using in order to reach that, a mean IPAP pressures of 22, more than 18. Look at the other study that have been shown also positive to decrease mortality. Not too different. They target physiologically their CO2 to decrease above somewhere in between 3 and 1 half to 7 millimeters of mercury. Look at the IPAP pressures that they are using, a mean IPAP pressures of 24 to 26. Again, we are looking for that IPAP pressures, but literally, the importance is not the pressure. The importance is your target. Your target is physiologically your CO2. So you're going to tell me, how do I do it? And that is where the key is start the conversation now. You know the background. You know who, when. Now we're going to say how. So the how comes with BiPAP-ST. This is where I put myself in my shoes. I'm receiving this patient from the intensive care unit after an exacerbation. I know that these patients are chronic COPDers. I know that has frequent exacerbations coming to my respiratory care unit in order to win off that bi-level. But I know that it will benefit from that bi-level to go home in the chronic step. So what I do is I do a lot of daytime acclimation. I do a lot because this is the only way that you can introduce these patients to bi-levels in high pressure intensity. So mass feeling, extremely important. I start with my devices in a BiPAP-ST. I started with 12 over four. And I put a backup respiration rate that is low with the hope that I'm going to not need the backup rate to maintain a minute ventilation. Because if that is the case, maybe just high pressures without the backup rate will be enough to qualify this patient for a RAD and send these patients with a further adjustment where they are being discharged. So if they tolerate that during the daytime, I start escalating by two centimeters of water. And I go into that 18 centimeters of IPAP. Again, I don't try to touch the backup rate. And I continue with the same inspiratory-expiratory ratio of one to three. Then comes the night. And then I place the capnography and I start my patient of BiPAP of 18 over four. And what I do is I place them on trascutaneous CO2 and I look it up if they can decrease their CO2. If they can decrease their CO2 by three to seven millimeters of mercury, then I'm done. I'm just done my first step into that high pressure slash high intensity intervention of these patients. Sure, if the oxygenation is great, we'll leave it there. If not, it's snoring or has some intermittent hypoxia, we start playing with the IPAP and then we may adjust it with oxygenation supplementation. But this is how we're doing it in that transition where I work about how to introduce these patients into these devices. So you're gonna say, well, now do I need a home mechanical ventilator? Do I need to break the bank? My account to put this patient, my taxes, to put this patient into a home mechanical ventilator when the patient doesn't need it. They need, they maybe need just a rat. And a rat again, these are with no, all the whistles of the, because you don't need it. This is a patient that they're relatively functional. So you'd need alarm. You don't need these independency with batteries. You don't need all the fancy volume mode. What you need is just qualifying for the BiPAP S or ST. This is where it's gonna get you your S. Your COPD plus the oxygenation is gonna get you to that E0470. That is the rat without a backup rate. So if you are okay doing that adjustment protocol that I have shown you with a backup rate of 10 and you have reached some of the decreasing CO2 just by the increasing the delta pressure, you don't need a backup rate. It has been proven in these patients that patients with high pressure and high intensity, meaning just high pressure versus those with high pressure with backup rate has equal outcomes. So you don't need necessarily backup rate. But however, if you have reached an IPAP that is too high and the patient cannot tolerate it, then unfortunately you need your backup rate to maintain that minimum ventilation and affect or impact your CO2. Then is when you need your backup rate. And when do you need your home mechanical ventilation? When your IPAP is so high that you cannot deliver it through a rat. When you need high oxygen supplementation or when you have persistent hypercapnia in these patients, then is when you get the big gaps. Then is when you get your home mechanical ventilation. So bottom line, high pressure is equally effective to the high intensity, meaning high pressure plus backup rate. Try to see if you can introduce your device during the daytime, acclimatize the patient, bring them as close as you can to the 18 and then follow them with some degree of CO2 to see that you can decrease that getting with IPAPs closer to 22 to 24. And then nothing needs to be perfect. You always can follow up your patient later and continue adjusting that. So acclimation is the key word in these patients if you're gonna treat it with high intensity or high pressure approach. That's it. So the last thing that he says is the one in red. The strategy on how to reach this goal, high intensity versus high pressures is probably not important. Important is your physiological target, your CO2. Thank you. All right, thank you everyone. I think we're a little over time. I don't know if there's another session at 8.30. It looks like there is. But we can answer questions offline for anyone who has questions. Thank you.

transitioning

home mechanical ventilation

chronic respiratory failure

devices used

patient populations

respiratory assist devices

home ventilators

clinical indications

acclimating patients

optimal ventilation