1. Pulmonary management of the neuromuscular patient Tim Short, MBA, BS, RRT
Welcome to “Pulmonary Management of the Neuromuscular Patient”.
This program has been approved for 1 hour of continuing education credit.

2. Objectives
Discuss management of patients with Amyotrophic Lateral Sclerosis (ALS)
Secretion removal
Noninvasive ventilation
American Academy of Neurology (AAN) practice parameters
Review the mechanics of airway clearance
Identify conditions that benefit from secretion mobilization and/or secretion removal
Intrinsic disease
Neuromuscular disease
Discuss techniques that may be used for airway clearance
List the objectives of the presentation.

3. Neuromuscular disease
Mucociliary transport is often normal
Amyotrophic Lateral Sclerosis (ALS) is a progressive neuromuscular disease that affects nerve cells in the brain and the spinal cord
Patients with neuromuscular disease have an impaired ability to eliminate secretions
It may be due to
Lack of respiratory muscle strength
Impaired ability to cough
Amyotrophic Lateral Sclerosis (ALS) is often referred to as Lou Gehrig’s disease. Lou Gehrig succumbed to the disease during the height of his baseball career. ALS is a progressive, degenerative disease that affects the nerve cells in the brain and spinal cord. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost and as a result the voluntary muscle action is also progressively lost.
Patients suffering from ALS or another restrictive lung disease have the inability to eliminate their secretions. This may be due to lack of muscle strength to generate a cough or an impaired ability to cough. Often with these types of patients the mucociliary transport is normal but increased sputum production id due to the inability to effectively eliminate secretions.
ALS is often referred to as
"Lou Gehrig's Disease"

4. ALS statistics 50 percent die within three years of disease onset
80 percent die within five years of diagnosis
10 percent live more than 10 years
NIV has prolonged the length of survival by treating the respiratory insufficiency.
ALS occurs in all races
1.5 to 1.0 ratio of men to women
Currently about 50% of the diagnosed ALS patients (those who do not use breathing support) die within 3 years of the disease onset. Eighty percent of the population will die within 5 years of diagnosis and 10% live past 10 years. Respiratory failure is the primary cause of death in ALS. However, respiratory failure can be prevented and treated. NIV has prolonged the length of survival of the ALS patient to greater than 10 years by treating the respiratory insufficiency caused by ALS. Most of those that are surviving greater than 10 years have chosen to use NIV when offered NIV therapy as an early option.
You can also see that ALS does not discriminate except that it affects slightly more men than women. 4

5. ALS Lung disease
When ALS affects the neurons that control the respiratory muscles, breathing becomes strained or weak
Begins with shortness of breath during exercise or at night
15 percent never have a breathing problem
In the past, diminished breathing was the usual cause of death.
Now, there are options.
Living with ALS: Adapting to Breathing Changes, 1997, ALS Assoc.
Let’s discuss a bit of the background information on how the disease affects the breathing system. ALS affects the neurons and when it reaches those neurons that control the respiratory muscles, breathing will become strained or weakened. This usually begins by shortness of breath occurring during exercise or at night while sleeping. Interestingly, 15% of the ALS population never has their breathing affected. But, remember this means there are 85% that may need ventilatory support.
Patients used to not get any ventilatory support at all or were trached and placed on a volume ventilator. Now there are noninvasive ventilatory support options.
In the past, diminished breathing was the usual cause of death, but this was before NIV was an option for those suffering from ALS. 5

6. Patient care strategy
Offer a comprehensive approach to the management of the ALS patient in the home.
Utilize a bi-level S/T ventilatory support system
Utilize compliance reporting systems and the device report
Provide additional therapies as needed
This allows for identification of patients early in the disease process, and for the provision of appropriate therapies for patient management throughout the course of the disease
It can be helpful to offer a comprehensive approach to the management of ALS patients in the home. The approach utilizes a ventilator for breathing support. This is followed up by downloading patient data through compliance reporting systems to assist in the management of the disease process. All of this leads to the possibility of being able to provide additional therapies if needed. This allows for identification of patients early in the disease process and for the provision of appropriate therapies for patient management throughout the course of the disease. 6

7. Lung disease Mucociliary transport is often compromised
Increased sputum production is a result of intrinsic lung disease
Patient has intact muscle strength for strong coughing
Patients suffering from intrinsic lung disease have the muscle strength for strong coughing but have thick secretions causing the mucociliary transport not to function effectively. This leads to mucus build up in the lower airways.

8. Groups at risk for retaining secretions
Intrinsic lung disease
Cystic Fibrosis (CF), COPD, bronchiectasis
Neuromuscular disease
ALS, muscular dystrophy, post polio, multiple sclerosis, spinal muscular atrophy
Spinal cord injury, stroke
Patients that are in danger of retaining secretions can be grouped into 2 disease categories. The first group are those with intrinsic lung disease such as cystic fibrosis, chronic obstructive pulmonary disease and bronchiectasis. The second group are those who have neuromuscular disease with muscle weakness or restrictive disease. Many neuromuscular diseases such as ALS, muscular dystrophy, and multiple sclerosis belong in this category. Both spinal cord injury and stroke patients also share this same risk. During this presentation we will focus primarily on patients with neuromuscular, or restrictive, disease.

9. Airway clearance cycle
Inhaled
irritant
Lung
damage
Inflammatory
response
Mucus production
Biochemical changes
Retained secretions create a vicious cycle of lung damage in a patient that is unable to clear them.
When an irritant is inhaled, the lungs’ defense mechanisms are set in motion.
An inflammatory response occurs, in which biochemical reactions take place (including release of white blood cells and water into the area), producing excess mucus to catch and eliminate the irritant or bacteria from the lung.
When the mucus cannot be mobilized and removed, secretions are retained.
Plugging of the airways leads to atelectasis (at-tel-eck-ta-sis - collapse of the airway) pneumonia and a ventilation-perfusion mismatch.
Inflammation and infections cause damage to the airways with changes in the lining of the airways. This injury is the beginning of a cycle in which your airways slowly lose their ability to clear out mucus.
Obstruction
Ineffective
airway clearance
Retained
secretions

10. Impact of ineffective cough
Approximately 90% of episodes of respiratory
failure within patients with neuromuscular
disease occur during otherwise benign upper
respiratory infections because of the inability to
clear the airways.
Tzeng AC. Bach JR. Prevention of pulmonary Morbidity for Patients with Neuromuscular Disease. Chest. November 2000:Vol. 118, No. 5;
The impact of not being able to cough effectively has a significant effect on those with neuromuscular disease or spinal cord injuries. This Chest paper documented that about 90% of respiratory failure is related to upper respiratory infections that normally would be controlled by an effective cough.

11. AAN practice parameters
The Care of the Patient with Amyotrophic Lateral Sclerosis (an evidence-based review)
American Academy of Neurology (1999)
Respiratory Work Group
Deborah Gelinas, MD
Edward A Oppenheimer, MD
In 1999, the American Academy of Neurology published the Practice Parameter: The Care of the Patient with Amyotrophic Lateral Sclerosis (An Evidence-Based Review). This review was written by a committee of well known physicians in ALS management. Assisting with the respiratory guidelines were Dr. Deborah Gelinas from California Pacific Medical Center and Dr. Tony Oppenheimer from Kaiser in LA. 11

12. AAN practice parameters
Five areas of investigation
Breaking the news
Symptomatic treatment
Nutrition
Respiratory insufficiency and mechanical ventilation
Advance directives and palliative care
The task force agreed to investigate 5 different areas: 1) informing the patient about diagnosis and prognosis, called “breaking the news” of ALS, 2) symptomatic treatment; 3) nutrition and decisions about the placement of a PEG or percutaneous endoscopic gastroscopy tube; 4) respiratory insufficiency and mechanical ventilation; and 5) advance directives and palliative care.
We are going to focus on the respiratory insufficiency and mechanical ventilation guidelines. 12

13. AAN practice parameters
Principles of ALS management
High priority should be placed on patient autonomy
Information is appropriately timed for decision-making
Address the full continuum of care
Advance directive discussions should be introduced and re-evaluated by the physician
To achieve the goal the task force developed several guiding principles or attributes of care:
High priority should be placed on patient self-determination or autonomy as an underlying assumption in the therapeutic relationship. Delivery of both information and care must take into consideration the cultural and psychosocial context of the patient and family.
2. Patients and families need information that is timed appropriately for decision-making, and delivered well in advance of major management crossroads, especially for respiratory care. Moreover, decision making is a dynamic process that may be subject to change as the disease becomes more severe.
3. The physician, in conjunction with other health care professionals, should address the full continuum of care for the patient with ALS, and nurture therapeutic interventions from diagnosis through palliative care for the terminal phase of the disease.
4. Discussions regarding advance directives should be introduced by the physician and reevaluated at intervals of no more than 6 months. Similarly, helping patients understand the issues to be faced in the terminal phase of the disease must be accomplished in a timely manner and empathetic fashion. 13

14. AAN practice parameters
Pulmonary measurements
Erect sitting vital capacity
Supine vital capacity
A decrease in VC to 50 percent is associated with respiratory symptoms
Nocturnal oximetry
Polysomnogram
The measurements that are most widely used in the ALS patient for early monitoring of respiratory status include the vital capacity, either erect sitting or supine. When the VC falls to a level of 50% of predicted VC, it is often associated with respiratory symptoms, but usually after these respiratory symptoms have already been recognized. NIV should have been initiated with the first appearance of respiratory insufficiency symptoms, but if it has not then it surely should be once the VC has reached 50% of predicted.
Nocturnal oximetry can also be useful in evaluating nocturnal hypoventilation.
A full polysomnogram is an alternative test in some patients. 14

15. Measuring cough strength
Maximum expiratory pressure
Isolates cough muscle strength
MEP of 60 cm H2O and higher has been shown to correlate well with the ability to generate adequate cough flows
Peak cough flow
Simple testing format
Peak flow meter and mask
Measure Peak Cough Flow (PCF)
Normal: 6-12 l/s or 360–720 l/min
Peak cough flow is used to measure the effectiveness of the cough because it captures the strength and ability of all the muscle groups involved.
Peak cough flow can be measured very practically by attaching a naso/oral mask to an inexpensive peak flow meter and having the patient cough into the meter rather than just blowing. PCF can also be measured by a pneumotachometer that might be used for simple spirometry.
Another trending tool that can be used to monitor the strength of a cough is maximum expiratory pressure. This maneuver obviously requires the ability to completely seal a mask or mouth piece but it does correlate well with the ability to clear secretions. Maximum expiratory pressures of 60 cm H2O or above have been shown to correlate well with the ability to generate adequate cough flows.

16. Ineffective cough
Respiratory muscle strength can deteriorate during respiratory infections
PCF between 160 l/minute but less than 270 l/min are also at risk
For this reason, a PCF of 270 l/min has been used to identify patients who would benefit from assisted cough techniques
Bach J, Ishikawa Y, et al. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 1997;112 (4):
It is important to note that persons with a neuromuscular deficit can weaken further during respiratory infections and those with peak cough flows greater than 160 l/min but less than 270 l/min may still be at risk as fatigue and further weakness sets in along with a respiratory infection. For this reason, the target pcf most often used to identify those who can benefit from assisted cough is a minimum of 270 l/min.

17. Ineffective cough PCF < 160 l/min
Increases risk of respiratory infection
Introduces risk of chronic lung damage as a result of recurring lung infection
Increases risk of pneumonia and resulting hospitalizations
A patient needs to be able to generate a minimum peak cough flow of 160 l/min for the cough to be effective. An ineffective cough leads to an increased risk of a respiratory infection. With recurring infections, the result is lung tissue damage. And, an ineffective cough can result in pneumonia requiring hospitalization.

18. Airway clearance therapies
In recent years, both new technologies and more advanced technologies have been developed to increase comfort and effectiveness of airway clearance for the majority of patients. Due to the number of products and techniques that are said to provide airway clearance, determining the most appropriate treatment can be confusing.
What to use?

19. Understanding airway clearance methods
Secretion removal
Techniques that mobilize and remove secretions from the lungs
Secretion mobilization
Techniques designed to loosen and mobilize secretions from the lower airway to the upper airway
It is critical for clinicians, patients and caregivers to understand the difference between the various methods of mobilizing and/or clearing secretions.
Secretion removal techniques mobilize secretions and remove secretions from the lungs.
Secretion mobilization techniques are designed to loosen and mobilize secretions from the lower airway to the upper airway by the use of vibrations.

20. Secretion clearing/removal techniques
Suctioning
Manually assisted cough (MAC)
Mechanical insufflation-exsufflation (MI-E)
Secretion clearance techniques include suctioning, manually assisted cough (MAC), and mechanical insufflation-exsufflation (MI-E).

21. Secretion mobilization techniques
Manual chest physiotherapy and postural drainage
External percussion and vibration devices
High frequency chest wall oscillation (HFCWO)
Aerosol therapy
Positive expiratory pressure devices (PEP)
Intrapulmonary percussive ventilation (IPV)
Effective cough needed to clear mobilized secretions
There are several different techniques that help mobilize and loosen secretions. Those include:
Manual CPT and postural drainage
External percussion and vibration devices
High frequency chest wall oscillation (HFCWO)
Aerosol therapy
Positive expiratory pressure devices (PEP)
Intrapulmonary percussive ventilation (IPV)
With all these techniques an effective cough is needed to clear the mobilized secretions.

22. Secretion mobilization techniques
“...are effective in mobilizing retained mucous secretions for patients with sick lungs (intrinsic), such as CF and COPD, although these devices will not help a patient whose main problem is impaired cough clearance.”
Finder, JD. Review of Airway Clearance Technologies RT Magazine July
Secretion mobilization techniques according to Dr Jonathan Finder are, “Effective in mobilizing retained mucous secretions for patients with sick lungs (intrinsic), such as CF and COPD, although these devices will not help a patient whose main problem is impaired cough clearance.”

23. Mechanical insufflation-exsufflation (MI-E)
Assists patients to clear retained secretions noninvasively
Applies a positive pressure to the airway (insufflation) followed by a rapid shift to a negative pressure (exsufflation) simulating a cough
May be applied by mask or mouthpiece or invasively via endotracheal or tracheostomy tube
Let’s discuss the clearing of secretions using mechanical insufflation- exsufflation or MI-E. This not a new technique; in fact, it has been used since the 1950’s for polio patients. It has been known through the years as the IN-Exsufflator but the modern version is called the CoughAssist. This therapy works nonivasively to removed retained secretions. A gradual hyperinflation is provided with positive pressure followed by a rapid shift to a negative pressure which simulates a cough. This therapy can be administered by mask, mouthpiece or invasively through a tracheostomy tube.

24. Indications for use for Cough Assist
Any patient unable to cough or clear secretions
effectively due to reduced peak cough expiratory
flow < 270 l/min
Contraindications
History of bullous emphysema
Known susceptibility to pneumothorax or pneumo-mediastinum
Recent barotrauma
Indications for MI-E include any patient having difficulty clearing secretions and unable to generate a peak cough flow of 270 l/m.
Contraindications are similar to other positive pressure devices and include history of bullous emphysema, pneumothoracies, and recent barotrauma.

25. Benefits of insufflation
Neuromuscular disease - reduced VC and Vt and an inability to sigh that result in developing atelectasis and pneumonia1
Provides normal hyperinflation - has been shown to combat loss of chest wall compliance and microatelectasis2
1 Estenne M. et al. Lung volume restriction in patients with chronic respiratory muscle weakness:
the role of microatelectasis. Thorax 1993:48(7):
2 Estenne M. et al. Chest wall stiffness in patients with chronic respiratory muscle weakness.
Am Rev Respir Dis 1983;128(6):
Insufflation helps to combat atelectasis and pneumonia by delivering sigh-like breaths as well as providing a range of motion type exercise for the improvement of external chest wall compliance.
Ref Resp. Care

26. Benefits of exsufflation
Flow simulates a natural expiratory cough flow (6-10 l/sec)
More effective than invasive suctioning since suctioning has been shown to miss the left mainstem bronchus 90% of the time1
Potentially eliminates the need for invasive suctioning
1 Bach Jr. Room For Imagination:Inspiratory and expiratory muscle aids. Advance. April 2006: 58-60
The benefits of exsufflation include generating 6-10 l/sec peak cough flow which is the highest of the assisted cough techniques. The need for invasive suctioning can often be eliminated.

27. Inhale + Exhale + Pause = Cycle
MI-E treatment
Inhale + Exhale + Pause = Cycle
Repeat cycle 4-6 times
A cough cycle consists of inhalation, exhalation and pause
4 to 6 cough cycles given in rapid succession.
The patient is then allowed to rest for 20 – 30 seconds.
A full treatment consists of repeating the above sequence 4 – 6 times.
The patient may not need 4 – 6 sequences to clear their secretions. If the patient has more secretions to clear, the patient must be rested for 5 minutes before resuming another treatment.
Rest seconds
Repeat sequence 4-6 times

28. Typical MI-E settings Pressures (positive and negative)
Start low, 10 to 15 cm H2O
Get patient acclimated to device
Increase pressures as tolerated, 35 to 45 cm H2O ideally1
Times (inhale, exhale and pause)
Small children: 1 to 2 sec
Adults: 2 to 3 sec
1 Gomez-Marino E et al.Mechanical insufflation-exsufflation. Pressure,volume, and flow relationships and the adequacy of the manufacturer’s guidelines. Am J Phys Rehabil 2002;81(8):
As far as machine settings, there are mainly 2 – set pressures and times. When first starting someone on the CA, it is recommended to start with low pressures of cm H2O allowing for an acclimation period.
Ideally we want to get to higher pressures
Higher pressures = higher peak cough flow = better secretion removal
Times listed here are good starting points and can be adjusted for patient comfort and treatment efficacy.
A pause can be set between each cough cycle for patient comfort and to aid with patient/machine coordination.

29. Settings
The goal of inspiratory pressure is to give the patient a good hyper-expansion
The goal of expiratory pressure is to replace a good expiratory cough flow
Pressures and times vary with each patient
It is important to remember the goals of using MI-E. Insufflation should be set to deliver a breath approaching maximal inspiratory capacity, this can be determined by physical assessment or auscultation. The exsufflation should be set high enough to provide PCF simulating a normal cough flow.

30. Settings
Important to work with patient to find settings which are both comfortable and effective
Studies have shown that therapeutic PCF may not be reached with MI-E expiratory pressures less than -40 cm H2O
Each patient’s pressures may be a little different based upon their lung compliance and resistance. Therefore we need to work with each patient to find comfortable settings that meet the clinical goals.

31. Clinical benefits
Mean peak cough expiratory flow rates of 21 patients with NMD studied
Unassisted ± 1.03 L/sec
Assisted cough ± 1.29 L/sec
MI-E & MAC ± 1.02 L/sec
(Normal PCF is 6-12 L/sec)
Conclusion: In-exsufflator cough machine improved peak cough expiratory flow rates
This study, published in Chest, shows the PCF improving with the use of an assisted cough, and significantly increasing when using both MI-E and MAC together.
Bach J. Chest 1993; 104:1553. 31

32. Therapy combination benefits
MI-E & MAC produced the most effective cough flows
This slide shows the results of the study on a graph. As you can see, the use of mechanical insufflation-exsufflation in combination with a manual assisted cough technique produces the highest peak expiratory cough flows in all 4 diagnosis groups.
Bach et al: Chest, 1993; 104:

33. Noninvasive ventilation
When working with patients who have neuromuscular conditions, such as ALS, it is important to understand the role of noninvasive ventilation and how to manage these patients throughout the progression of their disease.

34. ALS Lung disease during sleep
Weakened bulbar muscles can cause closing of the airway
Nerve and muscle functions relax during sleep causing under- ventilation
With complaints of morning headaches, lethargy, and SOB
Early recognition of weakening muscles during REM sleep by:
PSG
Overnight oximetry
Living with ALS: Adapting to Breathing Changes, 1997, ALS Assoc.
During sleep, there are a few things going on with the weakened muscles that will affect breathing. The bulbar muscles, or those that assist in swallowing mechanisms, may weaken and cause closure of the airway, similar to an OSA patient. Nerve and muscle functions relax causing the patient to be under-ventilated resulting in morning headaches, lethargy, and shortness of breath. These are the same symptoms as those of patients suffering from chronic respiratory insufficiency.
Initially, early recognition of weakening respiratory muscles can be noticed during REM sleep. The need for early NIV intervention may be recognized in the sleep lab with a PSG or with overnight oximetry. 34

35. This graphic came from a consensus paper provided by the ATS (American Thoracic Society) and ACCP (American College of Chest Physicians). The Consensus Conference paper was written concerning the implementation of NIV (non-invasive ventilation) for patients with hypoventilation. The graphic highlights that patients with hypoventilation will appear initially with sleep disturbances, followed by the addition of daytime symptoms and gas exchange issues that may occur during the day. As you can see, patients with alveolar ventilation problems may have them identified in the laboratory setting (if seen) by the center prior to daytime symptoms or complaints.

36. AAN practice parameters
Early indications of respiratory insufficiency
Dyspnea on exertion
Supine dyspnea
Fatigue
Disturbed sleep
Morning headaches
What are the early indications of respiratory insufficiency?
Early signs and symptoms can be overlooked if not watched carefully. Symptoms include dyspnea on exertion, supine dyspnea, marked fatigue, disturbed sleep (frequent nocturnal awakenings, excessive daytime sleepiness), and morning headaches.
NIV should be considered for the ALS patient with the symptoms of respiratory insufficiency.
Noninvasive ventilation should be initiated with the onset of symptoms 36

37. AAN practice parameters
NIV benefits to the patient
Improves the symptoms of hypoventilation
Improves quality of life
Increases survival by treating the respiratory insufficiency
Allows for decision making on more advanced care
Does NIV improve respiratory function or increase survival?
There are direct clinical benefits to the patient. Several studies in which we will go into more detail, indicate that NIV improves the symptoms of hypoventilation, thereby improving quality of life and increasing survival of patients with ALS.
The use of NIV can also allow for additional time for the patient to make decisions on more advanced care such as invasive ventilation.
We will go into some of these studies in more details in a moment. 37

38. AAN practice parameters
Recommendations
Be vigilant for hypoventilation symptoms
Offer noninvasive ventilatory support
Offer invasive ventilatory support
Respect the right of the patient to refuse therapy
When withdrawing ventilation, relieve dyspnea and anxiety
The AAN task force made these recommendations:
Be vigilant for symptoms indicating hypoventilation. Serial measures of pulmonary function (vital capacity) are recommended to guide management and to determine prognosis with the understanding that no single test can detect hypoventilation reliably.
Offer noninvasive ventilation ventilatory support as an effective initial therapy for symptomatic chronic hypoventilation and to prolong survival in patients with ALS.
When long-term survival is the goal, offer invasive ventilation and fully inform the patient and family of burdens and benefits.
In accordance with the principle of autonomy, physicians should respect the right of the patient with ALS to refuse or withdraw any treatment, including mechanical ventilation.
When withdrawing ventilation, use adequate opiates and anxiolytics to relieve dyspnea and anxiety.
Research has shown that guidelines such as these usually take upwards of 3 years for physicians to begin to embrace and implement the new standards. Many physicians are still not using these guidelines as some studies still show there is late implementation of NIV. This is a great opportunity to promote these guidelines to the ALS clinics and neurologists. 38

39. The committee strongly supports the use of mechanical insufflation-exsufflation in patients with DMD
Patients with DMD should be taught strategies to improve airway clearance and how to employ those techniques early and aggressively
Use assisted cough technologies in patients whose clinical history suggests difficulty in airway clearance, or whose peak cough flow is < 270 l/min and/or whose maximal expiratory pressures are < 60 cm H2O
The American Thoracic Society has issued this consensus statement encouraging the use of the MI-E with those diagnosed with DMD. It is important that patients are taught strategies to improve airway clearance early and aggressively. ATS recommends that assisted cough technologies be instituted when:
Patient has a clinical history that suggests difficulty clearing their airways
Peak flow is < 270 l/min
Maximal expiratory pressure is less than 60 cm H2O

40. Quality of life Study aim
Initiation of NIV earlier than current “standard of care” may provide additional benefits in terms of respiratory function and quality of life.
Jackson et al. A Prospective Evaluation of Pulmonary Function Studies and Symptoms of Hypoventilation in ALS/MND Patients. J Neurol Sci 2002;1610
A study by Carlayne Jackson from San Antonio was reported in the Journal of Neurological Sciences in 2002 and looked at the quality of life that was referred to in the AAN parameter paper. The aim of this study was to initiate NIV earlier than the current standard of care to see if it would provide additional benefits in pulmonary function and quality of life. “Standard of Care” meant not initiating NIV until the forced vital capacity FVC) dropped below 50% of predicted.
This study found that there was an increase in quality of life when NIV was started at 70% of predicted FVC rather than waiting until 50% of FVC. 40

41. Quality of life 13 patients
7 received early NIV intervention, 6 received NIV per existing standard of care
FVC was done in sitting or supine position
Pulse oximetry was performed
Dr. Jackson tested 13 patients. Seven received early NIV intervention and 6 received NIV in accordance with the existing standard of care. Notice that all study participants received NIV at some point in their disease progression with early intervention at 70% of predicted FVC and standard of care at 50% of predicted FVC. FVCs were performed in either the supine or sitting position, followed by pulse oximetry. 41

42. Quality of life Conclusions
To detect respiratory insufficiency, O2 saturation of less than 90 percent is a more sensitive indicator than FVC of 70 percent
Early intervention with NIV increased “vitality” subscale of the SF-36
NIV earlier than current practice may result in improved QOL
Dr. Jackson concluded that oxygen saturation of < 90% was a better indicator of respiratory insufficiency than the usually performed forced vital capacity of either 70% or 50% of predicted. The early intervention group had an increased vitality subscale of the SF-36. Dr. Jackson’s study also showed that NIV initiated earlier than current practice may result in an improved quality of life.
Overnight oximetry can be performed, and this information can be used by the physician to order NIV when appropriate.
The SF-36 was constructed to survey health status in the Medical Outcomes Study. It assess 8 health concepts: 1) limitations in physical activities because of health problems, 2) limitations in social activities because of health or emotional problems; 3) limitations in usual role activities because of physical health problems; 4) bodily pain; 5) general mental health; 6) limitations in usual role activities because of emotional problems; 7) vitality and 8) general health perceptions. 42

43. BiPAP in OSA with other co morbidities – AVAPS
Neuro-
Muscular
Disorders
OSA
AVAPS is the treatment of choice for patients presenting with chronic respiratory disease as well as a mixed SDB pathology.
AVAPS automatic ventilation modality allows normalizing ventilation with a target exhale Vt whereas the EPAP level maintains the UA open (treating OA).
The settable backup rate and the rise time allows better control and adaptation to different types of patients.
AVAPS
COPD –
Overlap
The majority of patients entering the sleep lab for treatment and titration will have straight OSA. With the data from Dr. Morgenthaler, 10 – 20% of patients entering with a possible diagnosis of OSA will end up with complex sleep disorders. That includes periodic breathing, and CPAP induced central apneas.
With the release of neuromuscular guidelines, patients with several forms of neuromuscular diseases will have issues with their sleep patterns as their disease progresses. As a result, they may enter the sleep lab for treatment of these sleep disorders. In addition, patients with COPD may also be sent to a sleep lab for treatment of a sleep condition.
Obesity Hypo-Ventilation
AVAPS
AVAPS

44. AVAPS Algorithm BiPAP Synchrony AVAPS
AVAPS is a ventilation device for patients with respiratory insufficiency
ST-T-CP modes can treat central apneas
AVAPS: Pressure Support adjustment looking at average tidal volume
PS increase or decrease maximum speed is 1 cm H2O/minute
AVAPS allow maintaining a target tidal volume
10 PM
7 AM
IPAP
Max.
+1 cmH2O/min Max
-1 cmH2O/min Max
IPAP
Min.
EPAP
Target Vt
This slide illustrates the AVAPS algorithm.

45. Patient tracing over time
Automatically adjusts IPAP to guarantee a target tidal volume
This demonstrates how the machine adjusts pressure over time to guarantee the tidal volume for the patient.

46. S/T compared to S/T + AVAPS
This is a sample of how the two devices can vary over time. The upper tracing is an indication of how a patient was titrated on a device and over a 3 month period of time - the device still has the same pressures. However, if you placed the patient on a AVAPS unit, you can see that over time the patient’s lung mechanics have improved due to the lowering of the IPAP pressures to maintain the same tidal volume. As a result, the patient was able to receive the appropriate care as their lungs improved.
Pressure support is progressively decreasing:
AVAPS follows patient’s needs and disease progression
The patient is getting better

47. Clinical benefits of AVAPS
Guarantee of ventilatory support during progressive ventilatory changes of the patient
Guarantee of ventilatory support during positional changes during sleep
Provides the assurance of a tidal volume within a bilevel system
Alarms to indicate that tidal volume is not being maintained
Clinical benefits of AVAPS.

48. Summary
Secretion mobilization techniques assist the mucociliary escalator, but they do not assist cough
Cough is the principle mechanism for clearing the airways
Patients who have an impaired cough mechanism require secretions to be removed
Initiation of NIV may improve quality of life and symptoms of hypoventilation in patients with neuromuscular disease
In summary,
Secretion mobilization techniques assist the mucociliary escalator, but they do not assist cough
Cough is the principle mechanism for clearing the airways
Patients who have an impaired cough mechanism require secretions to be removed
Initiation of NIV may improve quality of life and symptoms of hypoventilation in patients with neuromuscular disease