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INFORMATION FOR

"Sleep and Respiratory Management in Amyotrophic Lateral Sclerosis" Montserrat Diaz-Abad (09/29/2021)

October 11, 2021
  • 00:00Everyone and welcome to sleep seminar.
  • 00:03So I'm just going to start off with a few
  • 00:05reminders before I introduce our speaker.
  • 00:07So first these lectures that
  • 00:09are available for CME credit.
  • 00:10So just remember to text the
  • 00:12ID for the lecture to the Yale
  • 00:15cloud CME account this afternoon.
  • 00:17If you're not sure how to do that,
  • 00:19the information will be placed in the
  • 00:21chat periodically during the session
  • 00:23so you'll be able to see that if you
  • 00:25have questions during the presentation,
  • 00:27use the chat feature and we will
  • 00:29have time for questions at the end.
  • 00:31And also at the end,
  • 00:32if you'd like to unmute yourself,
  • 00:33that would be fine and then
  • 00:35otherwise keep your microphone muted.
  • 00:37I'd appreciate that.
  • 00:38So now it is my pleasure to
  • 00:40introduce today's speaker, Dr.
  • 00:43Monserrate D.
  • 00:44As a bad doctor Diaz.
  • 00:45A bad is associate professor of
  • 00:47medicine at the University of
  • 00:49Maryland School of Medicine.
  • 00:50She is director of the Pulmonary
  • 00:52rehab program there Co.
  • 00:54Director of the Ventilator weaning program.
  • 00:56She's also the Co director for the
  • 00:59Maryland ALS Certified Treatment
  • 01:01Center of Excellence.
  • 01:02And director of the Sleep Medicine
  • 01:05Fellowship program at university.
  • 01:07See Maryland ship ended middle
  • 01:10school at Intec in Santo Domingo,
  • 01:13from which she graduated Summa Coomb Laudy.
  • 01:15She completed her residency and
  • 01:18her PCSM Fellowship at Temple
  • 01:20University in Philly.
  • 01:22She then moved to University of Maryland,
  • 01:23where she spent much of her career.
  • 01:26She's been an active clinician and
  • 01:27educator with a scope of practice,
  • 01:29including the medical ICU,
  • 01:31pulmonary konsult services,
  • 01:33Sleep Medicine service,
  • 01:34and the ventilator, weaning and rehab unit.
  • 01:36She has numerous.
  • 01:37Publications in the areas of sleep,
  • 01:39sleep,
  • 01:39disordered breathing and non
  • 01:41invasive ventilation,
  • 01:42among many other activities.
  • 01:44She is a member of the Health and
  • 01:46Public Policy Committee for the ACP,
  • 01:47Maryland chapter.
  • 01:48She is a pulmonary advisor for the
  • 01:50Maryland Virginia DCLS Association
  • 01:52and she is a member of the
  • 01:54Ventilator Subcommittee for the
  • 01:56Northeast LS Research Consortium.
  • 01:57Additionally,
  • 01:58she's a recipient of several clinical
  • 02:00grants for the care of patients with a LS,
  • 02:02so we are pleased and delighted
  • 02:04to have doctor Diaz a bad.
  • 02:05Join us today to discuss sleep.
  • 02:07And respiratory management in LS so welcome.
  • 02:12Thank you very much, Doctor
  • 02:14Hilbert for the introduction.
  • 02:16I will proceed to start.
  • 02:20And dumb. So I I would like to talk
  • 02:23about sleep and respiratory management
  • 02:26enamor trophic lateral sclerosis.
  • 02:28And this is a disclosure side slide.
  • 02:34So they overview of amber traffic
  • 02:36ladders closest to begin with,
  • 02:38then pulmonary assessment and management
  • 02:41will proceed to talk about sleep disorders,
  • 02:45treatment of respiratory failure with
  • 02:47non invasive and invasive ventilation
  • 02:49and a little bit about multidisciplinary
  • 02:52clinic management in this disease.
  • 02:54So as a very brief overview,
  • 02:57LS is a syndrome with symptoms and signs
  • 03:00of degeneration of upper and lower
  • 03:03motor neurons leading to progressive,
  • 03:05unrelenting weakness of a most
  • 03:08muscle groups in the body.
  • 03:11Death due to respiratory failure
  • 03:13failure follow some in two to
  • 03:16four years after symptom onset.
  • 03:18Although there is a small subset 345
  • 03:21percent that made survive at decade
  • 03:23or more depending on the final.
  • 03:25Cognitive dysfunction has been increasingly
  • 03:28recognized in this population is being
  • 03:31noted to be as high as 50% in some groups,
  • 03:343 to 5% develop frontotemporal type dementia,
  • 03:38and this is a factor because it
  • 03:41affects a compliance with an ID,
  • 03:43for example.
  • 03:45These are just some general numbers.
  • 03:47They vary year by year and by by publication,
  • 03:51but in general mean onset is
  • 03:53middle age close to older age.
  • 03:56There's some male to female ratio
  • 03:58of two to one familiar cases are
  • 04:01probably now closer to 10% because of
  • 04:04increasing increasingly recognizing
  • 04:06of a different jeans and more general
  • 04:10typing being done in any given year,
  • 04:13approximately 30,000 people.
  • 04:15I'm leaving with a LS in the United
  • 04:17States and roughly about 5000 new
  • 04:20patients are diagnosed annually,
  • 04:22so 5000 for the whole country that
  • 04:24it doesn't give a lot of opportunity
  • 04:27for exposure to these patients.
  • 04:29A mean time from onset of symptoms
  • 04:32to diagnosis is 13 to 18 months.
  • 04:35It can be very long.
  • 04:36A lot of these patients can get
  • 04:38different procedures and neck
  • 04:41decompression believe that they
  • 04:43have cervical psygnosis and.
  • 04:46EMT procedures,
  • 04:47etc.
  • 04:48So it's it's really challenging
  • 04:50because it's a diagnosis of exclusion.
  • 04:54So it why are pulmonary and sleep
  • 04:57physicians involved in the care?
  • 04:59As mentioned,
  • 05:00patients die from respiratory
  • 05:02failure and this is a there's a nice
  • 05:05series done by caution in in France
  • 05:07where they took 100 consecutive
  • 05:09patients that were admitted to the
  • 05:12hospital that had a LS and died,
  • 05:14hospitalized and they did out of seat
  • 05:17on their on their hundred patients and
  • 05:21they confirmed that 71% of them died.
  • 05:24From pneumonia,
  • 05:25both community acquired hospital acquired
  • 05:28minority and aspiration pneumonia,
  • 05:30so This is why both sleep pulmonary
  • 05:34management is very important.
  • 05:36The major test that we do for a LS
  • 05:40monitoring is spirometry with forced
  • 05:42vital capacity and this shows in a less
  • 05:47restrictive ventilatory defect and.
  • 05:49Which is a surrogate of
  • 05:51respiratory muscle weakness.
  • 05:53Both the absolute FBC and its slope of
  • 05:56decline are good predictors of disease
  • 05:59progression and survival sexually.
  • 06:01The more the most validated predictor.
  • 06:05However,
  • 06:05there is great variability in in the
  • 06:08decline and but within a particular
  • 06:11patient they it does tend to be linear,
  • 06:14at least during some period of the disease.
  • 06:17So essentially some people
  • 06:18declined very quickly and some.
  • 06:20People decline a faster and
  • 06:22I'll show a graph showing that
  • 06:24average decline is about 3.5%,
  • 06:27but it varies widely.
  • 06:30So they they graph below.
  • 06:32Here they figure below.
  • 06:33I like in particular.
  • 06:35There are many studies
  • 06:36about every see showing,
  • 06:37but this one since there were
  • 06:39not many patients you can see
  • 06:42every individual measurement.
  • 06:43Every dot of the vital capacity
  • 06:46measured up to 70 months.
  • 06:49And as you can see you do get
  • 06:52these patients that are very slow
  • 06:54progressing and then you get patients
  • 06:56that in three months in six months
  • 06:59go from normal vital capacity to.
  • 07:01To essentially being on the ventilator.
  • 07:04And I will refer a little bit
  • 07:07to the to this study in a couple
  • 07:09more slides from this study.
  • 07:11I liked very much.
  • 07:12Essentially this was done by bit,
  • 07:14AKA at all in in Italy they took 25
  • 07:18patients and ALS and they did every
  • 07:21possible test under the pulmonary test,
  • 07:24respiratory mechanics, etc.
  • 07:26And it's very,
  • 07:27very thorough and has a lot of
  • 07:30information and for example,
  • 07:32they found that the desired outcome,
  • 07:35six months or Bible.
  • 07:36Patients who survive tended to
  • 07:38have a higher bio capacity than the
  • 07:41patients that were dead by six months.
  • 07:43Of course,
  • 07:44they were the patients who
  • 07:45were there were older.
  • 07:46This is a small group,
  • 07:47but this is just a sample of
  • 07:49what has been shown in multiple
  • 07:51studies that they initial vital
  • 07:53capacity is a predictor of how
  • 07:56long the patient will leave.
  • 07:58Uhm,
  • 07:58then other measurement besides
  • 08:00parameter that we do is measurement
  • 08:02of respiratory muscle air pressures.
  • 08:05This is a usually maximal
  • 08:07inspiratory pressure.
  • 08:08Maximal expert Tori pressure and one
  • 08:11of the ways to measure inspiratory
  • 08:14pressure can also be done with the Smith,
  • 08:18which is essentially a it's done
  • 08:21with a plug in the nose and the
  • 08:24person sniffs forcefully and
  • 08:26it gives a value that is.
  • 08:28It has similar value to a
  • 08:31maximal inspiratory pressure,
  • 08:32or sometimes people refer to it
  • 08:35as negative inspiratory force and
  • 08:38the advantage of that is that
  • 08:40it allows persons people have a
  • 08:43or a weakness to be able to do
  • 08:45it if they are not able to do a.
  • 08:47A C / A over the mouthpiece and
  • 08:50it has been shown to have a
  • 08:53great correlation with the gold
  • 08:56standard which is transparent,
  • 08:58pragmatic pressure.
  • 08:59But which requires the insertion
  • 09:01of an aesopi yelling gastric balloon.
  • 09:04And here is an example of many of
  • 09:08how the initial measurement of
  • 09:11a muscle strength depending on
  • 09:14what the initial measurement is,
  • 09:16and they and they decline the
  • 09:19initial measurement and the decline,
  • 09:20they survival.
  • 09:21Essentially,
  • 09:22it depending if you have a higher number,
  • 09:25you live longer and this is power
  • 09:27of over five years,
  • 09:29there's a.
  • 09:32This is the same study I from VITACCA,
  • 09:36but in this one I want to focus on and
  • 09:39this part of the respiratory mechanics,
  • 09:41which is the a respiratory
  • 09:44rate over tidal volume.
  • 09:45What in the ICU you hear measure mentioned
  • 09:48as a risky or rapid shallow breathing index?
  • 09:52I think this is sometimes on the recognized
  • 09:56but and I have not seen this studied
  • 09:59in in in as much detail in any other.
  • 10:03Publication essentially,
  • 10:04if the diaphragm is weak,
  • 10:07then the breathing becomes shallower,
  • 10:09so the person has to breathe faster and the
  • 10:13reason is as the breathing becomes shallower,
  • 10:16the proportion of alveolar ventilation,
  • 10:18total ventilation decreases.
  • 10:20So if normal Dead Space is 30% for
  • 10:25someone who has a tidal volume of
  • 10:28500 that maybe 150CC's if that person
  • 10:30now has a shallow breath of 300.
  • 10:33That same that same Dead Space
  • 10:35doesn't change it's 150,
  • 10:37but now it's 50% of the breath,
  • 10:39so that person has to increase
  • 10:41their respiratory rate to maintain
  • 10:43our bill or ventilation.
  • 10:45Using a diaphragm that is already weak
  • 10:48to begin with and and very simply if
  • 10:51even if not measured with a because
  • 10:54I don't bottom, is not available.
  • 10:56Even the respiratory rate can
  • 10:57be very telling.
  • 10:58Here was not significant.
  • 11:00The numbers are very small, but you can
  • 11:03see at a difference of 28 versus 20.
  • 11:05So I think someone with a LS that is
  • 11:08breathing fast and shallow is very very.
  • 11:11It's something to take into
  • 11:13consideration and start an Ivy.
  • 11:15Earlier, even if they're not hypercapnic.
  • 11:18So it in full every sleeper,
  • 11:20by the way,
  • 11:21so this virus across the board I
  • 11:23ask Doctor Hilbert mentioned II.
  • 11:26I'm part of the ventilation
  • 11:28committee in this Niels Association
  • 11:30we had a meeting in 2018.
  • 11:32There were 21 of us and we started
  • 11:34raising her hand. Who does this?
  • 11:37Who does porometry all?
  • 11:3821 razor hands. Who does a Maple met?
  • 11:4214 Razor Hands who does speak off low?
  • 11:45Three raised her hand.
  • 11:46Who does a sleep study?
  • 11:49Regularly for razor hands.
  • 11:50Who does oximetry and on and on so
  • 11:54essentially after vital capacity
  • 11:56and make a map. It's one third one.
  • 12:004th 1/5. So essentially there's no.
  • 12:03There's no, uh,
  • 12:05there's no consistency because there's
  • 12:09very little data beyond the vital
  • 12:13capacity and and probably a map.
  • 12:15So everyone does it differently.
  • 12:17I'm I'll mention what?
  • 12:19We do we do a upright for spinal capacity.
  • 12:22We do mapping map and we do a pic awful.
  • 12:26We also used to do arterial blood gas
  • 12:28during every clinic visit with when
  • 12:31patients were non invasive ventilation.
  • 12:33We have transition we gotta end
  • 12:36title CO2 monitor so we do that in
  • 12:39every clinic and we infrequently
  • 12:41now do nocturnal oximetry or PSG
  • 12:44but we do it if necessary.
  • 12:47We may do some questionnaires.
  • 12:50We also do as essentially
  • 12:52when I'm evaluating them.
  • 12:54I do a sleep slash pulmonary evaluation
  • 12:57as the usual things about breathlessness
  • 13:01and also non restorative sleep,
  • 13:04daytime sleepiness etc and on exam I
  • 13:09always try to.
  • 13:11Have them with very little close.
  • 13:13Maybe it's being shared or not even
  • 13:15that to see their chest movement.
  • 13:17Put my hands on the chest,
  • 13:19make sure that see if the diaphragm is did.
  • 13:22The chest is moving.
  • 13:23Is it moving bait more lower that the
  • 13:25diagram is moving and there's expansion?
  • 13:28Or is he moving more based on
  • 13:30the accessory muscles on top?
  • 13:32So that's very helpful.
  • 13:34Also ask the patients took off.
  • 13:36Sometimes patients go and
  • 13:37and and and that's very.
  • 13:39That's a very important to note.
  • 13:41A week off,
  • 13:43are you having problems with secretions?
  • 13:47Second life flat.
  • 13:48Paradoxical movement is very telling.
  • 13:51Sometimes I it's very efficiently
  • 13:53so now wheelchair.
  • 13:54We don't get them to the exam table,
  • 13:55but if they are mobile, it I.
  • 13:58I've seen patients that we lay
  • 13:59down and 30 seconds later,
  • 14:01or saying I cannot breathe,
  • 14:03so that's because of orthopnea.
  • 14:06So one thing that I want to
  • 14:08mention here is the supine for
  • 14:10spiral capacity or vital capacity,
  • 14:13depending on which measurement
  • 14:14you do slow or or or force,
  • 14:17this is very telling of a suggested not
  • 14:21only of a diaphragmatic weakness for my LS,
  • 14:25but diaphragmatic weakness
  • 14:27from any situation.
  • 14:28So typically, on a normal quote,
  • 14:32unquote, healthy, let's say they they.
  • 14:36Drop is up to 10% once you start
  • 14:40getting into a less group in
  • 14:42this series can be it was 15%,
  • 14:45but it actually can be 2025.
  • 14:48One thing that because we don't
  • 14:50do the the supine regularly.
  • 14:53One thing that I've found very
  • 14:56suggestive is when patients up
  • 14:58orthopnea unless they have another
  • 15:00reason like heart failure etc.
  • 15:02That is very, very suggestive of a of a.
  • 15:06A very weak diaphragm in this
  • 15:09clinical situation,
  • 15:11and it's almost a I would say not equivalent,
  • 15:15but very similar and these are patients.
  • 15:17In this case,
  • 15:18the daytime fatigue is more sleepiness,
  • 15:20even higher, so it's we do.
  • 15:25Is sometimes do so prime,
  • 15:27but it's more on the patient with
  • 15:30undiagnosed disneya that already
  • 15:32has had PFTS and CT and everything,
  • 15:35and you're trying to find the cost
  • 15:37once they have their diagnosis,
  • 15:38we go more by symptoms such as
  • 15:41orthopnea and the upright in vital capacity,
  • 15:43but I this is again another example of
  • 15:47resources is someone has the resources.
  • 15:50It would be ideal to do U.S.
  • 15:52Open supine vital capacity.
  • 15:53I want to talk a little bit.
  • 15:55Cost maneuver because these patients,
  • 15:59even though we may do only only sleep
  • 16:02and not pulmonary for a some group of people,
  • 16:06is sometimes I've seen people
  • 16:08who are not pulmonary,
  • 16:10that they prove anology.
  • 16:11Sent them the LS patients,
  • 16:13or neuromuscular to manage their noninvasive,
  • 16:16not all the time.
  • 16:17But sometimes they they they.
  • 16:20I have seen it happen.
  • 16:21Wanna talk a little bit about the
  • 16:23cough maneuver it during inspiration
  • 16:25person takes a they take a breath
  • 16:28in and then the glottic a glorious
  • 16:31closes for about .2 seconds.
  • 16:34During that Glottic closure
  • 16:36the abdominal muscles contract
  • 16:38forcefully and there is a buildup
  • 16:40of pressure because of that
  • 16:42contraction with a closed glottis.
  • 16:45Then the glottis opens here and
  • 16:47you see this huge flush of air.
  • 16:50And we did speak call flow here at
  • 16:52this spike baby baby that's a normal
  • 16:55cough and then you have a drop of flow
  • 16:58in a drop of pressure and while their
  • 17:01strongest cough occurs at high levels
  • 17:04of total lung capacity 85 not 90%.
  • 17:08You can even have a very forceful
  • 17:11cough with even low lung volumes
  • 17:14because of the effectiveness of this
  • 17:17maneuver and this this this huge flows.
  • 17:21And they share.
  • 17:22They move the mucus from the
  • 17:26Airways and expel it.
  • 17:28And this is a patient with a LS
  • 17:30where they do it spirometry and then
  • 17:32they just had them serially cough.
  • 17:35As you can see even at the lower lumbar
  • 17:37and you can still generate a High
  • 17:40Peak flow pressures and and and that
  • 17:42are effective and then you see the pics here.
  • 17:45This is someone who has moderate bulbar
  • 17:48impairment and this is someone that.
  • 17:50Taskbar paralysis so it they it's.
  • 17:54This has a lot of implications
  • 17:56for management and survival.
  • 17:58We measure pickup flow usually without
  • 18:03peak expiatory me in flowmeter like
  • 18:06the the the asthma once normal is
  • 18:10360-2840 liters per minute or even higher.
  • 18:13It has been estimated that at least a
  • 18:16pic of low 160 years permit is needed.
  • 18:19These values are not very precise because
  • 18:21as you know this virus depending on height,
  • 18:24predictive value etc.
  • 18:26But it's a broadcast inmate and.
  • 18:29In the US,
  • 18:304 prescribing compass sistance you do
  • 18:33not need to measure the pickup flow.
  • 18:37You'll only need a report from the
  • 18:38patient that they have problems
  • 18:40coughing with the appropriate diagnosis,
  • 18:42such as a LS.
  • 18:43But I there's some places I've seen some
  • 18:46provinces in Canada that they actually base,
  • 18:50went to prescribe it on measuring
  • 18:52the peak of flow we you can fit
  • 18:55the pickoff flowmeter with a mask.
  • 18:58If the patient has evolved a weakness.
  • 19:00We actually do some of our PFTS with masks.
  • 19:03If they patient cannot have a,
  • 19:05uh,
  • 19:06a good seal,
  • 19:07we have stopped using the flow meter
  • 19:10because these were individual for
  • 19:12patients and what we tell they slipped a.
  • 19:15The PFT Technologies is to actually just
  • 19:19do an expert do have them do a cough
  • 19:23and record the spirometry maneuver.
  • 19:25So then the peak exploratory flow
  • 19:27of that maneuver is a peak of flow.
  • 19:30So that is A and then we if we multiply
  • 19:33by 60 you get 4 liters per minute,
  • 19:36for example 4 liters per second.
  • 19:39That is 200. And 240 liters per per minute.
  • 19:44So it's very it's very easy and
  • 19:47and do not have to do.
  • 19:50They are additional costs of the flow meter,
  • 19:53so the main mechanisms in maneuvers that
  • 19:58can help with cough is essentially A2 fold.
  • 20:05Without any equipment without any
  • 20:08mechanical advanced equipment,
  • 20:10so this is for example that case
  • 20:11of a 49
  • 20:12year old woman, very low vital
  • 20:14capacity of 10% predicted and
  • 20:17pick off low 43 liters per minute.
  • 20:20We mentioned at least 160 we need
  • 20:22and the patient was trained to beths
  • 20:24breast stack with an essentially with
  • 20:27an AMBU bag or sleep ventilator was
  • 20:30able to take two to three breaths,
  • 20:32one on top of each other to
  • 20:34build the total lung capacity.
  • 20:36And by doing that they.
  • 20:39Was able to take in 2.5 liters
  • 20:42and the peak of flow increased
  • 20:45to 120 liters per minute.
  • 20:47So on this patient there was
  • 20:49a second maneuver was added,
  • 20:52which is a manually assisted coughing cough,
  • 20:55which is it has different ways of doing it,
  • 20:58but it's essentially almost like
  • 21:00our handling maneuver pushing on
  • 21:02the top on the top of the abdomen
  • 21:04at the same time that they called,
  • 21:06and this was able to increase to 283 liters.
  • 21:09For a minute.
  • 21:10So sometimes we educate their
  • 21:12patients about that.
  • 21:14Just be aware,
  • 21:15try to take deep breaths and
  • 21:17push on on the top of your.
  • 21:19If you're having trouble coughing
  • 21:20until we get you there, help.
  • 21:22We need so secretion management you know,
  • 21:26just swallow solation can
  • 21:28be used if we just cheer,
  • 21:30say the mucus and from the smaller
  • 21:34Airways and centers in the larger Airways.
  • 21:39I we don't use it a lot because we use
  • 21:42the next device that we were talking about,
  • 21:45but it it has a used in cases
  • 21:47of patients that have a lot of
  • 21:50purulent secretions that not just
  • 21:52have a LS or it could be someone
  • 21:55that has moderately week off that
  • 21:57needs some help but cannot tolerate
  • 22:00the the insufflator extra later.
  • 22:02However,
  • 22:03I there is some concern about someone
  • 22:06who has zero cough or almost 0.
  • 22:10'cause moving a lot of secretions
  • 22:12into the central airway and and and
  • 22:15and having difficulty breathing.
  • 22:17So this may not work, especially in advance.
  • 22:21In in very advanced patients,
  • 22:23but may help in mild to moderate patients.
  • 22:27There's a one random miles randomized
  • 22:30trial of a A this device in a LS.
  • 22:35Patience very small.
  • 22:36You'll see you'll get used to seeing
  • 22:38this very small number of patients in this.
  • 22:4119 versus 16.
  • 22:43Untreated,
  • 22:43and the treated patients said
  • 22:46that they had some breathlessness.
  • 22:48They caught up at night,
  • 22:49more maybe because secretions
  • 22:51were mobilized but not expelled.
  • 22:53UM and I declining breathlessness,
  • 22:57no significant change in forced
  • 23:01vital capacity.
  • 23:02I've gone to a lot of conferences,
  • 23:04they they a lot of people don't use this,
  • 23:08some do, but not.
  • 23:09It's not.
  • 23:10It's not like the first line
  • 23:12therapy for these patients.
  • 23:14I know it's in my arsenal,
  • 23:17but do not use it in the beginning.
  • 23:20Initially in most patients what we do use
  • 23:23is the mechanical insufflator exit later,
  • 23:26the brand name,
  • 23:27the only one available in EU S is
  • 23:30cough assist and what this does.
  • 23:32This applies positive than
  • 23:34negative pressure to the Airways
  • 23:36synchronized with the breathing.
  • 23:38So essentially when people think
  • 23:39about all these patients on
  • 23:41by level 15 / 5, well imagine these patients
  • 23:44are getting 40 centimeters of water,
  • 23:46for example, of positive pressure.
  • 23:49It seems the machine synchronize
  • 23:51it with inspiration, and then when
  • 23:53the patient switches to expiration,
  • 23:56it becomes dramatically drops to minus 40.
  • 24:00For example, it can go usually 30 to 50.
  • 24:0330 to 60.
  • 24:05And this essentially sucks everyone.
  • 24:07Everything up out of the airway
  • 24:10into the upper airway.
  • 24:11Very effective.
  • 24:12UM, this cycle Times Now there
  • 24:15is there not no need to memorize
  • 24:18because now the machines come very
  • 24:21synchronized with the breathing.
  • 24:23The Micmac in the past you had
  • 24:25to time it a little bit more.
  • 24:27We recommend that patients use this
  • 24:29twice a day and then as needed.
  • 24:32This is a an example of a study
  • 24:35that was done.
  • 24:37This included all types of neuromuscular
  • 24:39patients and they compare the pick
  • 24:42a card flows generated by the
  • 24:44different techniques I mentioned.
  • 24:45One of them is on assisted.
  • 24:47They first on assisted them
  • 24:49with the breath breath stacking,
  • 24:51then the breath stacking plus assisted,
  • 24:54and then the extra later insulators.
  • 24:57So clearly an advantage advantage in
  • 24:59terms of of flow. I do want to caution.
  • 25:02There's a Cochrane analysis that
  • 25:05reviewed this and did not find there's
  • 25:09a clinical impact on on whether it
  • 25:12was mechanical or just assisted,
  • 25:15meaning that reaching a certain
  • 25:17level may be a good enough.
  • 25:20The advantage of this is that it
  • 25:22doesn't require this caregiver attention.
  • 25:25It does require caregiver attention
  • 25:26to put it on the mask,
  • 25:28but it doesn't require beyond the
  • 25:30initial training and much more
  • 25:32advanced training.
  • 25:33So it can be simpler to do,
  • 25:34and we tend to do that.
  • 25:36The other advantage is my first
  • 25:38slide with the Bitter Cup paper
  • 25:40I I want to mention that another
  • 25:43potential advantage of the
  • 25:45mechanical insufflator X of later,
  • 25:47which is that that initial
  • 25:50positive pressure or 40 year,
  • 25:52essentially doing a long recruitment and
  • 25:54you're helping a lot with atelectasis,
  • 25:57and these patients tend to
  • 25:58have a lot of collectors,
  • 26:00especially at the long basis
  • 26:02because of the diaphragmatic.
  • 26:03Weakness and also a collector sees in
  • 26:05general because of the shallow breathing,
  • 26:08so one of the things that really helps that,
  • 26:12uh,
  • 26:12even though I don't prescribe it
  • 26:14for atelectasis because it's not
  • 26:16reimbursed I as soon as the patient
  • 26:18has a little bit of trouble coughing
  • 26:21that it that it's the patient says yes.
  • 26:23Sometimes I have trouble coughing.
  • 26:25I immediately prescribe it
  • 26:27because it will help.
  • 26:29Also with atelectasis this is in that group.
  • 26:33It decreased compliance.
  • 26:35Patients who survived had normal lung
  • 26:38compliance and you know the compliance.
  • 26:41They they lower your compliance the less.
  • 26:45Able joy you are able to stay
  • 26:47less ability to stretch the
  • 26:49lungs so they increase work of
  • 26:51breathing so they more compliant.
  • 26:53Your long chest wall etc.
  • 26:55It it it decreases the work of
  • 26:58breathing and patients feel better.
  • 27:00So this is a UM,
  • 27:02an added benefit that I that I think it is
  • 27:06very helpful.
  • 27:07So what are factors affecting sleep?
  • 27:10And I will then later concentrate on sleep
  • 27:13disorder breathing but in general imagine.
  • 27:15I diagnosis and getting a diagnosis that
  • 27:18has so many significant consequences.
  • 27:22Patients may have some problems and
  • 27:23they get excited. These problems
  • 27:25get exacerbated or they are new.
  • 27:27Depression, anxiety, insomnia,
  • 27:29pain, muscle cramps, contractors.
  • 27:32A big one is choking with his excessive
  • 27:36airway secretions for bulbar patients.
  • 27:39Inability to move in bed I just
  • 27:42position confinement in bed in activity.
  • 27:46Cognitive dysfunction.
  • 27:47You name it and there is A and usually
  • 27:50there's two three factors in every patient.
  • 27:53Sometimes there's one, but it's a big
  • 27:56community combination of factors,
  • 27:58so in so it's very challenging
  • 28:02to manage these patients and we.
  • 28:05As mentioned,
  • 28:06have to be aware that we are aware that
  • 28:09there are multiple concomitant factors,
  • 28:11so we have to address them one by 111,
  • 28:16measure that.
  • 28:16I have found incredibly useful
  • 28:19for these patients is recommending
  • 28:21a wedge and caribair elevation,
  • 28:24and we can start.
  • 28:25This can be bought online anywhere 20,
  • 28:28thirty, $40.
  • 28:29I do find it more useful than
  • 28:32the pillows because the pillows
  • 28:33move throughout the night,
  • 28:35but this is very useful because this
  • 28:38helps a lot with the airway secretions.
  • 28:42That the patients app and sometimes that's
  • 28:44the only complaint that patients are.
  • 28:47I wake up all the time,
  • 28:48coughing and gagging a because of
  • 28:51secretions going into the airway.
  • 28:53So I wedged.
  • 28:54We start with 20 degrees or 30
  • 28:57and it's incredibly helpful.
  • 29:00And actually it's also helpful.
  • 29:03Sometimes I don't want it to be that helpful.
  • 29:05'cause I want the patient to be a
  • 29:07non invasive ventilation and then the
  • 29:09patient is like all since I started
  • 29:11the wedge I sleep more better.
  • 29:12Much better,
  • 29:13I don't have any shortness of breath.
  • 29:14Is sleeping because it elevates and
  • 29:16and it helps with the orthopnea.
  • 29:19So in a way, it it it.
  • 29:21It's I think in any in every way.
  • 29:23It's very helpful and we of course
  • 29:26when they qualify a hospital bed
  • 29:28is very difficult to do behavioral
  • 29:31interventions in these patients
  • 29:33because this what you think about
  • 29:35stimulus control.
  • 29:36It's not like they can get out of
  • 29:39bed and whenever they want sleep
  • 29:41restrictions, well you know what?
  • 29:43The caregiver uses a Hoyer lift and
  • 29:46puts them in bed at 8:00 o'clock
  • 29:48and before the daytime nurse leaves
  • 29:50so they are there in bed from 8:00
  • 29:53to 8:00 o'clock at night to 8:00
  • 29:55o'clock in the morning,
  • 29:56whether they're sleeping or not.
  • 29:57So it's very challenging and,
  • 29:59and that's something that of interest
  • 30:01in that at some point would like
  • 30:04to learn more of what behavioral
  • 30:07interventions are done on other
  • 30:08conditions with limited mobility,
  • 30:10like stroke, etc. These patients.
  • 30:13One thing that we do address that is.
  • 30:17Easily addressable, although not easily done,
  • 30:20is that we really focus on avoiding
  • 30:22the daytime sleeping. These patients.
  • 30:24They say they they keep the red partner,
  • 30:26their family awake at night,
  • 30:28but it's because they're napping in
  • 30:30and out during the day. So we really,
  • 30:32really stress at the very least,
  • 30:34don't sleep during the day as
  • 30:35much as possible, of course,
  • 30:38UM, better adjustments,
  • 30:40occupational therapy can help
  • 30:42their son a different types of
  • 30:46blankets and different surfaces.
  • 30:49ETC. UM medications we sometimes
  • 30:53use it as dual intent.
  • 30:56So for example,
  • 30:57if we have a patient that is having
  • 31:00increased salivation and is a little
  • 31:03depressed and also is having some
  • 31:07trouble initiating sleep sometimes
  • 31:09amitriptyline may be a good option.
  • 31:12Will have a anticholinergic
  • 31:13effects dry their secretions,
  • 31:15maybe cause a little bit of sleepiness.
  • 31:17Maybe treat a little bit their depression.
  • 31:19So sometimes they the same with clonazepam.
  • 31:22Sometimes people have these contractors
  • 31:24and stiffness and hypertonicity
  • 31:27and I have a little bit of anxiety.
  • 31:29A little bit of insomnia.
  • 31:31So in you have to be careful
  • 31:34for respiratory depression,
  • 31:35but that's I mean small doses
  • 31:38of benzodiazepine's etc.
  • 31:39They usually well tolerated and we do
  • 31:43tend to be and probably more generous with,
  • 31:46the sleep medications in the LS
  • 31:48clinic that in sleep clinic.
  • 31:50So if you cannot say well,
  • 31:53try this and see me in three months and
  • 31:55then we'll reassess in three months.
  • 31:57They may be there so you wanna
  • 31:59give improve the quality of life
  • 32:01and make them sleep better.
  • 32:02So I I do have a lower threshold
  • 32:05to give a a more medications.
  • 32:07Not immediately.
  • 32:08I necessarily I do want to address
  • 32:11some behavioral interventions
  • 32:12such as the daytime sleep.
  • 32:15So sleep disorders in a LS.
  • 32:17There's very little study.
  • 32:20This is a study of 76 patients.
  • 32:23For example,
  • 32:24they found RLSA was more frequent.
  • 32:27I haven't found this this
  • 32:29big difference in my clinic,
  • 32:31but there's more studies to be done.
  • 32:34And of course, this is 41 patients.
  • 32:36They they, of course you know.
  • 32:39I say, of course,
  • 32:39maybe 'cause I'm used to it,
  • 32:41but they have this PSG evidence
  • 32:43of decreased quality of sleep and
  • 32:46then then you go down to the the
  • 32:49lower numbers where this study.
  • 32:51Of 22 patients versus for a behavior
  • 32:55disorder where we took two patients or
  • 32:59with events, sleep without or atonia.
  • 33:01I mean there's very little information
  • 33:04and we need to do a more study about this.
  • 33:07But there's some indications of
  • 33:10some disorders being more frequent.
  • 33:12We did a study in our clinic,
  • 33:1443 newly diagnosed patients compared
  • 33:17to 43 controls we administer.
  • 33:20The Pittsburgh.
  • 33:21The Back Depression inventory inventory.
  • 33:23Two,
  • 33:24the Epworth and our functional
  • 33:26rating scale for a LS.
  • 33:28First of all we did find a small
  • 33:31increase in in the in in worse sleep quality.
  • 33:35Higher Pittsburgh in the LS
  • 33:38patients compared to control.
  • 33:39We also if we define poor sleep
  • 33:42quality as more than five the
  • 33:45score 63% of the LS patients had
  • 33:48poor sleep quality compared to 37.
  • 33:50In terms of sleepiness,
  • 33:51we didn't find as a I mean there was a.
  • 33:55Slightly slight difference,
  • 33:57but not this the this
  • 34:01incredible difference.
  • 34:02However you do see the individual
  • 34:05patients that are sleepy,
  • 34:06not just the major difference
  • 34:08of the group as a whole.
  • 34:09We did find and these are what
  • 34:12is consider a weak correlation.
  • 34:15But we did find a correlation between
  • 34:18the depressive symptoms and the
  • 34:20Pittsburgh and the inability to turn
  • 34:22in bed which we actually found very.
  • 34:25Trusting and these associations we
  • 34:27we were happy to even find a weak
  • 34:30association because as mentioned,
  • 34:32there's so many factors interfering
  • 34:34with sleep.
  • 34:35That is, it will be in a small group
  • 34:38difficult to find one factor that
  • 34:41has a really strong correlation.
  • 34:44And as an example of the inability to
  • 34:46turn in bed, this is a Pittsburgh score.
  • 34:50UM, in patients that have little
  • 34:53to no trouble turning in bed.
  • 34:55Compared to those that cannot turn in bed,
  • 34:58and as you can see, Chris,
  • 35:00a significant difference in this in the
  • 35:03in the score and that got me thinking,
  • 35:07wow, you know you turn all the time.
  • 35:08These patients sometimes are putting bad
  • 35:11on their back and they cannot move anyway.
  • 35:14They have to.
  • 35:15Wake up their their bed
  • 35:17partner to have them move.
  • 35:19Sometimes they don't want to wait
  • 35:21because I don't wanna bother them,
  • 35:23so it's it's.
  • 35:24It's very challenging for bed partner
  • 35:26and if patient so this is the best
  • 35:30study I have found on on PSG and ALS.
  • 35:35And actually I think this is
  • 35:37like the the reference study.
  • 35:38Before that there were there
  • 35:41was a scattering of multiple
  • 35:43studies with 1020 patients.
  • 35:45This I think.
  • 35:46Answered a lot of questions 'cause
  • 35:47this private studies.
  • 35:49This prior studies had a lot of
  • 35:52contradictory information.
  • 35:52In essence they always say was
  • 35:55found in 46% of patients nocturnal
  • 35:58hypoventilation in 40 in both conditions.
  • 36:01In 22.
  • 36:02If you want to say mild OSA,
  • 36:05we don't care about for mother and severe,
  • 36:09it was 23% of patients.
  • 36:11Central sleep apnea are
  • 36:13not clinically significant,
  • 36:14different to what was.
  • 36:15Reported in a couple of small case series.
  • 36:18One thing that I found very interesting
  • 36:20is that one third of the patients
  • 36:23will battle capacities that may be
  • 36:25considered normal or almost normal.
  • 36:28Had nocturnal hypoventilation,
  • 36:30but they, interestingly,
  • 36:31half of the patients with a vital capacity
  • 36:35less than 50 had nocturnal hypoventilation,
  • 36:39but almost hardly not so it's it's it's.
  • 36:42It's kind of I.
  • 36:43I would be for this.
  • 36:45I would have.
  • 36:46Assumed a higher a higher number
  • 36:48with people with low vital capacity,
  • 36:50but this is not the only measurement that
  • 36:53that comes in these patients and this
  • 36:56kind of mentions that they pulse oximetry.
  • 36:59It's helpful and we do it sometimes,
  • 37:02but it's not as sensitive
  • 37:05as measuring ventilation.
  • 37:07This is another study.
  • 37:09Twenty patients with OSA 22
  • 37:12without always say they found,
  • 37:14and this is the months to death
  • 37:16to death or tracheostomy.
  • 37:18The group had always say was 15.9 months.
  • 37:21The group that noise, say 26 months,
  • 37:24they there is no explanation.
  • 37:27They authors thought.
  • 37:28Well,
  • 37:29maybe this is a different pheno
  • 37:31type etc. But it's just kind of
  • 37:34intriguing and suggestive and needs
  • 37:37more larger groups to confirm.
  • 37:40This is one of those things that with the
  • 37:42with the REM sleep and non REM sleep.
  • 37:44When you have this acrobatic weakness
  • 37:46and then you lose your accessory
  • 37:48muscles it becomes very dramatic.
  • 37:50This is a chest and abdomen and the
  • 37:52flow and you can see in non REM pretty
  • 37:55good and as soon as they go into R.E.M.
  • 37:58A pretty pretty pretty decreased
  • 38:01movement because the accessory
  • 38:03muscles are are lost so this is a
  • 38:06this is something to keep in mind.
  • 38:08I have seen some.
  • 38:10Patients that sometimes they
  • 38:12may get that diagnosed with OSA,
  • 38:15but they don't have always say
  • 38:17they just have this.
  • 38:18They have decreasing in in the
  • 38:20flow and their studies at and it
  • 38:23gets marked as a high problem.
  • 38:25But so I I'm sometimes like we said at
  • 38:2923% may have severe or moderate OSA,
  • 38:31but sometimes I'm a little bit
  • 38:33suspicious when I see someone
  • 38:35that only has hypopneas only has
  • 38:38the hypopneas during RAM.
  • 38:40And there's no snoring, so it to keep IE.
  • 38:44So I sometimes.
  • 38:46Maybe a little bit more in, and even if.
  • 38:52With other parts may put in
  • 38:54OSA as a diagnosis.
  • 38:56I do make a comment.
  • 38:58Listen, this may be likely.
  • 38:59Hyperventilation,
  • 39:00based on what we saw.
  • 39:02This is a so that person knows not
  • 39:05to go in and slap her as sleep
  • 39:08up of 15 centimeters of water.
  • 39:10Uhm, this is a very nocturnal oximetry.
  • 39:13Seeing that another way of showing
  • 39:16the dramatic drop in in ventilation
  • 39:18or oxygenation that happens during
  • 39:21rain sleep very remarkable in March,
  • 39:24and because of what I mentioned as you know,
  • 39:26REM sleep tends to occur this
  • 39:28later part of the night.
  • 39:30So This is why split night PSG is not
  • 39:34ideal for these patients in this area.
  • 39:37For this group of 47 patients, 40.
  • 39:403% had an incomplete split night
  • 39:42and the major reason was the
  • 39:45absence of significant ramps lead.
  • 39:47Conversely,
  • 39:47you can argue that 57% it was effective,
  • 39:51but it's just this is the new romasco,
  • 39:54the allesandro muscular patients in general.
  • 39:57If you wanna get a complete picture and
  • 39:59you cut down the study here and to it,
  • 40:02you may not get this full picture of
  • 40:05what the patient is doing at night.
  • 40:08This doesn't this just FY I
  • 40:11that they sometimes it's.
  • 40:12It's tricky to know when to put a
  • 40:15feeding tube and an and if you wait
  • 40:18too late you may find yourself in
  • 40:21a position of essentially having
  • 40:23to do a feeding tube on non
  • 40:26invasive ventilation and we don't.
  • 40:28We don't want to do that so if the
  • 40:31pulmonary function is declining
  • 40:33it may be appropriate.
  • 40:35If the patient wants to to pick it.
  • 40:37If they are going to want it
  • 40:39anyway to put their feet into
  • 40:41sooner rather than later,
  • 40:43that doesn't mean going to an extreme
  • 40:45of putting them on all the initials.
  • 40:47But if they if it's declining,
  • 40:48it's very helpful to consider that because
  • 40:51it decreases the risk of the procedure,
  • 40:54we have transitioned a lot of our
  • 40:56patients to do it while you're
  • 40:59graphically inserted gastrostomy by
  • 41:01interventional radiology because they
  • 41:03need less sedation because they don't
  • 41:05need a EGD to get it done, they do.
  • 41:08They can do it with topical
  • 41:10anesthesia and minimal sedation,
  • 41:12so this has allowed us to do it in more
  • 41:16advanced patients that we will they
  • 41:19gastroenterologist and anesthesiologists
  • 41:21feel concern about a doing it.
  • 41:24Endoscopic Lee.
  • 41:27Hey, criteria for non invasive
  • 41:28ventilation in a less I mean it
  • 41:31improves survival and quality of life.
  • 41:34You can see here hypercapnia nocturnal
  • 41:37hypoxemia and make a more negative than
  • 41:40minus 60 or vital capacity less than 50.
  • 41:44Good news is you only need one so
  • 41:48that's very helpful is simply to get
  • 41:50non invasive ventilation for these
  • 41:52patients that for COPD for example.
  • 41:54Uhm, this is the only randomized trial
  • 41:57done in a lesson in Ivy it was 22 versus 19.
  • 42:02They picked some patients with some
  • 42:05mild to moderate and mild hypercapnia,
  • 42:08is some orthopnea and by vital capacity
  • 42:10you can see 40s and 50s and they
  • 42:14showed an improved survival in these
  • 42:16patients compared to standard care
  • 42:19which is the web when they divided their
  • 42:22groups between Barber and on Barber.
  • 42:24Meaning non non severe bulbar they
  • 42:27they saw their benefit specifically
  • 42:29in the number number patients or mild
  • 42:33to moderate a Barber involvement
  • 42:35in the severe bulbar involvement.
  • 42:38They didn't see a benefit and this
  • 42:40is the only randomized trial that
  • 42:42will ever be done.
  • 42:43An observation ull see is studies.
  • 42:46Retrospective studies.
  • 42:47Subsequent to this have shown that
  • 42:51patients even with a billboard
  • 42:53involvement can benefit.
  • 42:55It may be that they have worse tolerance,
  • 42:58but that they do still benefit.
  • 43:01Does the recommendation from the guidelines.
  • 43:03All the guidance is and do not stop
  • 43:07recommending and I be just because
  • 43:09of bulbar involvement give, give.
  • 43:11Give it a try on everyone and then see.
  • 43:15This is just what you would expect
  • 43:18that T0 is patients without an ID
  • 43:21and then they did a PSG on an Ivy
  • 43:24and essentially you see the the
  • 43:27A all the sleep parameters,
  • 43:29improving oxygenation parameter
  • 43:31respiratory rate, decreasing, etc.
  • 43:38And that study from the prior page.
  • 43:40These patients you save apps for 62 or 65.
  • 43:44I just like guys.
  • 43:45Sometimes people ask what protocol is used.
  • 43:49This is what they use in this one.
  • 43:51They tend. You tend to use low E pack
  • 43:57because of a exploratory muscle weakness
  • 44:00and only use higher E Patton four.
  • 44:03If you have a clear obstructive sleep apnea,
  • 44:06the iPod ranges.
  • 44:07Whatever you need to generate your
  • 44:10goal title, volume and in this
  • 44:11study you can see they did that,
  • 44:14but then it's adjusted.
  • 44:15The respiratory rate was two less
  • 44:18than what they found on the PSG
  • 44:20they title volume range 6 to 10.
  • 44:22Most people use eight,
  • 44:24we sometimes use seven to initiate
  • 44:26patients because we find that it's
  • 44:28the it's better tolerated than 8,
  • 44:31specially if they have been breathing
  • 44:33rapid and shallow league for a while,
  • 44:35and then we can titrate wait nasal mask.
  • 44:39And then follow up as needed.
  • 44:41It's just a graph of the elapse.
  • 44:45Outpatient titration we see patients
  • 44:47will download the data every visit.
  • 44:49We check pulse oximetry and entitle or
  • 44:53ABG with our transition more to entitled.
  • 44:57Because of the convenience and
  • 44:59less patient discomfort and
  • 45:00depending on what the patient says.
  • 45:02Hey, how are you doing? How do you feel?
  • 45:04Do you feel well supported?
  • 45:05Do you want a bigger breath?
  • 45:06You want a smaller bread or larger breath
  • 45:09and then we'll make adjustments based on
  • 45:12comfort and feedback from the patient.
  • 45:14And they it P CO2 and we can
  • 45:17increase the backup rate,
  • 45:18the title volume,
  • 45:20the pressure to support increased
  • 45:22daytime used, change the mask.
  • 45:24And as the last we saw,
  • 45:25bring them back into the lab.
  • 45:28Masks are very critical in this patient
  • 45:30population is very challenging because
  • 45:32they need sometimes help putting the mask on.
  • 45:35They have drooling.
  • 45:37Then they have drooling,
  • 45:39but then their mouth is open,
  • 45:40so if you do nasal everything,
  • 45:42scapes and even the chinstrap doesn't help.
  • 45:45But then, if you put a full face mask,
  • 45:47then they they have the saliva accumulated,
  • 45:49so it's very challenging they mentioned
  • 45:51can be challenging weight loss,
  • 45:53etc.
  • 45:56Uhm, this is a a an example this
  • 46:01case small case series.
  • 46:02Two patients with masks and dumb and
  • 46:06they full face versus up pillows and
  • 46:09essentially in one patient it did not
  • 46:12make a difference in related to the
  • 46:15airway and another another patient
  • 46:17it it did so it's I think it's that's
  • 46:20something that already has been
  • 46:22studied significantly in always say but.
  • 46:25I do want to point that out that the.
  • 46:30They they they,
  • 46:32it's important if possible to do a
  • 46:35nasal a nasal mask over an oral mask.
  • 46:39But of course or a mask if not.
  • 46:41And actually I'm going to stop
  • 46:43sharing for one second and I'm gonna
  • 46:45show you a video of one minute of a
  • 46:49of this paper and sometimes seeing
  • 46:53things is is helpful, so here it is.
  • 47:05In this video,
  • 47:06two patients diagnosed with Amy Tropic,
  • 47:09Lateral sclerosis can be seen there
  • 47:12using my invasive ventilation while
  • 47:14rate monitored through video for us.
  • 47:18Nearly ventilated with two different mask
  • 47:21interfaces for a nasal and intranasal masks.
  • 47:25Since that one is unmasked
  • 47:27prices on the mandible,
  • 47:29causing retraction and diminishing
  • 47:31the size of the upper airway,
  • 47:34disparities can be seen in size of the upper
  • 47:37airway and in the positions of the mandible,
  • 47:40tongue, soft palate and highly drunk.
  • 47:43Future studies month.
  • 47:45OK, so let me share back my presentation.
  • 47:50So just something to keep in mind
  • 47:53come and we did a case study of a
  • 47:56LS in we did our first A we use
  • 47:59a box instead of vibes because
  • 48:01that's what we have in our lab.
  • 48:03We did our first case study
  • 48:06in first titration.
  • 48:07This patient a lesson in May of 2012.
  • 48:10Special decline pretty quickly
  • 48:12over over several months from 79%
  • 48:15to 38% and we did a sleep study.
  • 48:19Patient did not have sleep apnea.
  • 48:21What she had was a sleep hypoventilation.
  • 48:24We did a datacard analysis over 15
  • 48:28weeks and the one thing to show is
  • 48:32that this device was manufacture
  • 48:34about 10 years ago.
  • 48:36So it's the algorithms have
  • 48:38improved significantly.
  • 48:39But I do want to point out that it every
  • 48:42week the DATACARD showed an 8 hi hi.
  • 48:45Whereas on the PSG and they
  • 48:47titration it didn't.
  • 48:48So they even this is not
  • 48:50necessarily the case.
  • 48:52Now, because as mentioned,
  • 48:53the machines are improved,
  • 48:54but to keep in mind that sometimes the
  • 48:58hyperventilation events may be mistaken,
  • 49:00and to be if something doesn't make sense,
  • 49:03sometimes it's better to bring
  • 49:04the patient to the lab.
  • 49:06This patient had progressive in
  • 49:08a fluctuating but increased use
  • 49:11of the ventilator everyday.
  • 49:13You know, more banks,
  • 49:15ventilators with a very familiar with.
  • 49:17We sometimes tend to use
  • 49:20regular bilevel with.
  • 49:22A box or backup rate and then usually we
  • 49:25use a bipap avaps or it can be there.
  • 49:28I bops and then when the patient progresses
  • 49:32from night time to daytime use we we
  • 49:36switch to a more advanced ventilator.
  • 49:39We need to have discussions with
  • 49:41the patients about end of life.
  • 49:43This is a a group of 24 patients and
  • 49:46only four were extubated and with a
  • 49:49LS so it's not that it's impossible.
  • 49:53You get extra later.
  • 49:54We have said expectations that it's not.
  • 49:56Don't expect to be excavated.
  • 49:58More likely than not, and this is
  • 50:01a invasive mechanical ventilation.
  • 50:04Essentially,
  • 50:05the point is like the last bullet,
  • 50:09.8 patients were placed
  • 50:10without their consent.
  • 50:12508 would not want to undergo it again,
  • 50:14so there are a lot of patients
  • 50:16that are put on the ventilator.
  • 50:17Essentially, if you wanted,
  • 50:19you are happy with it most of the time,
  • 50:2288%.
  • 50:23But if you did not want
  • 50:25it and you're put on it,
  • 50:27I I large proportion are not happy.
  • 50:30Although there are three out of eight were,
  • 50:31so it's important to have this
  • 50:34discussion that pragmatic pacing
  • 50:36to a randomized trial showed
  • 50:39worsening mortality with it.
  • 50:41Uh, I think very few people
  • 50:44are doing this anymore,
  • 50:46and so to finalize last couple of slides,
  • 50:49and we want to maximize quality of care,
  • 50:52discussed options of.
  • 50:53End of life and a peg tube, etc.
  • 50:57Early advanced directives.
  • 51:00Early referral to Hospice as needed.
  • 51:04Very prevention, vaccinations.
  • 51:06I tried to get everyone to
  • 51:09get vaccinated dental care.
  • 51:12Very important it we had them when
  • 51:14they come initially to the clinic.
  • 51:16Hey, if you haven't been to
  • 51:18the dentist goal decreases
  • 51:19pathogenicity of the bacteria.
  • 51:21Also still be terrible to have
  • 51:23someone 24 hours on a Bipap.
  • 51:25And then having to get a carries
  • 51:27a with the with the with the
  • 51:29mask on which we've had that.
  • 51:31So we're trying to prevent that important
  • 51:33to secure our primary care provider
  • 51:36'cause this clinic is not available 24/7.
  • 51:39Action plan on respiratory infections.
  • 51:42Introduction to the Heimlich maneuver we
  • 51:45have to monitor the caregivers and burnout.
  • 51:48Social work is very
  • 51:49important for these patients.
  • 51:51We have our Maryland ALS clinic.
  • 51:54They want me to say now.
  • 51:56A center of excellence.
  • 51:57So, uh, this is a center of excellence.
  • 52:01We have multiple specialties,
  • 52:03specialist at CD,
  • 52:04patient in the clinic that wants bolded,
  • 52:07and we have close relationship
  • 52:10with their other specialists.
  • 52:12And this is our our, our group.
  • 52:14And this is one day that we were
  • 52:17in clinic and and took a picture.
  • 52:19Thank you very much.
  • 52:20Let me stop sharing.
  • 52:31Thank you very much.
  • 52:32That was absolutely outstanding, really.
  • 52:34An excellent overview of of
  • 52:35how to manage these patients,
  • 52:37so I appreciate that for
  • 52:39everyone who's participating,
  • 52:40please feel free to come put a question
  • 52:42in the chat or unmute yourself.
  • 52:44I'd be happy to relay those questions
  • 52:47so I I'm very interested in your at
  • 52:50the beginning of your presentation.
  • 52:52You mentioned that older study
  • 52:53that talked about the frequency,
  • 52:55title, volume ratio,
  • 52:56and I have the same impression as you do,
  • 52:58that that's actually quite helpful that.
  • 53:01To look at that,
  • 53:02when we see the patients initially
  • 53:03and then later on on their downloads,
  • 53:05you know title,
  • 53:06volume and frequency are both are both
  • 53:09available and we can actually measure that.
  • 53:11And Lisa Wolf had a publication
  • 53:13looking at Vaps versus you
  • 53:15may be familiar with this,
  • 53:16but I'm sure you are vaps versus
  • 53:18bilevel a number of years ago,
  • 53:20published in Annals of ATS looking weather,
  • 53:22frequency title,
  • 53:23volume ratio was helpful in
  • 53:24deciding what mode might be better,
  • 53:26and it was a retrospective review,
  • 53:27but that sort of shed some light on that.
  • 53:30Is that something you're using to?
  • 53:31Follow patients you know when you're
  • 53:32looking at these downloads and deciding
  • 53:34if patients are doing well is that
  • 53:35one of the things that you look at.
  • 53:37Yes, uh, my listen, I are in that same
  • 53:40meals committee so we were doing that
  • 53:42so and I I read her paper. She's very.
  • 53:45She's like I probably one of the people
  • 53:48that knows the most about the topic.
  • 53:50Yes, I I. I look at it if I see a
  • 53:53high respiratory rate and for example
  • 53:55I have a gold title volume of seven,
  • 53:58I will increase it to wait.
  • 54:00And I also or maybe increase
  • 54:02it even at a touch more.
  • 54:05And I also look at the triggering.
  • 54:07So I don't want this when these patients.
  • 54:10If you someone has oh HS or whatever,
  • 54:12it doesn't matter if they
  • 54:14trigger on their own,
  • 54:14they may be even more comfortable,
  • 54:16but in this LS patients mouth to
  • 54:19moderate is not as significant.
  • 54:21But when they're advanced,
  • 54:23having them have to trigger every
  • 54:26single breath as a work workload.
  • 54:28So I look at the triggering and
  • 54:30if I see that they're triggering
  • 54:337080% of their breasts,
  • 54:34I will increase the respiratory
  • 54:36rate to try to lower that.
  • 54:38You cannot make it 0 because then
  • 54:39you run the risk of the patient.
  • 54:41Then the patient calls you that they're
  • 54:43getting a breath when they don't want it,
  • 54:44but I try to know where that,
  • 54:47ideally to 20% a roughly so that
  • 54:50they trigger less and make a
  • 54:52little bit less work of breathing.
  • 54:55So that's another.
  • 54:56It's just like a it's so much stuff II
  • 54:58that that that that we could talk about,
  • 55:01we could.
  • 55:01We could sit for an hour and compare notes.
  • 55:05I agree I agree.
  • 55:06UM, other questions.
  • 55:07I'm not seeing anything in the chat yet.
  • 55:10One thing you know,
  • 55:11you mentioned the comment.
  • 55:12The other thing that struck me,
  • 55:13you know it is compared to other disorders.
  • 55:16You know, the the current laundry list of
  • 55:17hoops to jump through to get these patients.
  • 55:19Or you know, a respiratory assist
  • 55:22device is certainly easier than
  • 55:24COPD or hypoventilation syndromes.
  • 55:26They only have to fit one of the four,
  • 55:28but at the same time you bring up
  • 55:30the point that these patients may
  • 55:32look pretty good during the day
  • 55:33and only have hypoventilation.
  • 55:35Based on nocturnal,
  • 55:37transcutaneous CO2 and not oximetry.
  • 55:39And of course the technical
  • 55:41expert panel is looking.
  • 55:42It has added that now as a possible
  • 55:45addition to the to the qualifying
  • 55:48qualifying criteria to be able to put
  • 55:50patients on you initiate mechanical
  • 55:52ventilation in these patients,
  • 55:55whether that will go through or not.
  • 55:56I don't know,
  • 55:57but what are your thoughts there?
  • 55:59Oh, absolutely.
  • 56:00I'm Karen Johnson that I see here.
  • 56:03I know she's one of one of those panels I.
  • 56:06And I if all these rules were changed,
  • 56:09it would like it would make everyone's
  • 56:11life easier and patient care in improve.
  • 56:14They really do not make sense.
  • 56:16I sometimes their consoles by
  • 56:18pulmonologist just because they don't
  • 56:20know the rules and and you do it.
  • 56:23But I even I sometimes have to
  • 56:25look them up after after all these
  • 56:27years for a particular patient.
  • 56:29So it's looks so your completed
  • 56:32by Doctor Hilbert.
  • 56:34I look so forward to these changes.
  • 56:36I know they're good people involved,
  • 56:38and so yes before I'm done.
  • 56:41I do wanna do a little shout out to
  • 56:44Shruti Katapa that rotated with us in
  • 56:46Sleep Medicine I I know her well and I'm
  • 56:49so glad that she is on faculty there.
  • 56:52She was a absolute delight and
  • 56:55pleasure to have on rotation for sleep.
  • 57:00We agree, we agree completely.
  • 57:04Alright, other questions.
  • 57:05Anybody feel like unmuting
  • 57:06themselves and ask yourself or
  • 57:08drop something in the chat for us?
  • 57:13I know we have some pediatric
  • 57:14people on the panel too.
  • 57:16I don't know if they have questions
  • 57:18about transitions of care we were
  • 57:19talking about that a little bit before.
  • 57:21We would have time for maybe
  • 57:22one one or two more questions.
  • 57:33So so yeah, well we were talking about that.
  • 57:35We do is come when a pediatric pediatric
  • 57:38patient wants to be transition to adult.
  • 57:41We have a three year overlap
  • 57:43where they pediatrician and
  • 57:44adult take care of the patient.
  • 57:46So at 18 the first adult base it is
  • 57:49done and sometimes with me I I take
  • 57:52a lot of care of the non invasive.
  • 57:55And that patients will tricks
  • 57:57and special needs to,
  • 57:58and so they do that visit with me.
  • 58:01And then they, or with another
  • 58:03specialist if it's not in vain invasive.
  • 58:06If it's not ventilation related
  • 58:08or city related and then and then
  • 58:10they go back to the pediatrician
  • 58:12then they come back to me.
  • 58:14Then they go back to the pediatrician
  • 58:16alternating and then at 21 just
  • 58:18before 21 they have that final
  • 58:20visit with the pediatrician.
  • 58:21And then I take over their care
  • 58:23and that works fantastically.
  • 58:25Because it gives a 3 year period
  • 58:28for the patient too in the family
  • 58:30to get used to that.
  • 58:32So it it it has worked really
  • 58:34well if if we also because we
  • 58:36have very good communication with
  • 58:37our pediatric colleagues.
  • 58:40I see Doctor Craig Kenny Perry,
  • 58:42so he just unmuted himself.
  • 58:43So feel free to ask her question.
  • 58:46Oh no, I just wanted to say
  • 58:47that I I enjoyed the talk,
  • 58:48I I'm the neuromuscular pulmonologist.
  • 58:51For for kids at our institution, and.
  • 58:53It's interesting you're talking about
  • 58:55the OR nasal versus nasal masks,
  • 58:57and are are kind of biases.
  • 58:59We always use nasal masks. Just
  • 59:00'cause of a aspiration
  • 59:02risk anyway, and certainly
  • 59:05it many not so much in the MDA clinic
  • 59:08population, but a lot of other patients
  • 59:10on non invasive ventilation may have
  • 59:13significant cognitive limitations or
  • 59:15other reasons that we just prefer to
  • 59:17use a nasal mask even though we're
  • 59:19dealing with the mouth leak and.
  • 59:20The transition issue is authority.
  • 59:23One you know, we have a lot of providers
  • 59:25who are invested in making it work,
  • 59:27but the system stuff is pretty
  • 59:30challenging in terms of.
  • 59:32You know, we've been talking a
  • 59:34little bit about maybe running.
  • 59:36You know actually Co.
  • 59:37Locating
  • 59:38for some visits with pediatric and
  • 59:40adult providers 'cause it has been
  • 59:42challenging and Jenna we can certainly
  • 59:45talk about this offline as well.
  • 59:47If you guys have any progress on your side.
  • 59:49We are meeting for awhile
  • 59:50talking about it, but it's it's.
  • 59:53It's hard 'cause Pediatrics and
  • 59:55the adult world have different
  • 59:56styles and just want everybody to.
  • 01:00:00Feel comfortable to transition.
  • 01:00:01But yeah, I was super
  • 01:00:02interesting talk. Thank you.
  • 01:00:04Yeah, it's it's very challenging.
  • 01:00:06Everyone I talked to it's challenging
  • 01:00:08in every institution unless they
  • 01:00:10have some pre stablished system
  • 01:00:12and it's going to get increasingly
  • 01:00:14challenging because these patients
  • 01:00:16are surviving more and more absolutely
  • 01:00:19right. Absolutely well I think we're at time.
  • 01:00:22I've really enjoyed your talk,
  • 01:00:23it's excellent and I'm sure you may
  • 01:00:25get some later questions that pop up.
  • 01:00:28People might email you but but
  • 01:00:29thank you again for your time.
  • 01:00:30It's really been outstanding.
  • 01:00:32Thank you. I it was my pleasure to
  • 01:00:34be here and please feel free to share
  • 01:00:37my email if anyone has any questions.
  • 01:00:40Great thank you. OK bye bye bye.