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

October 11, 2021

ID7003

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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.