BiPAP & Ventilation
What Is a BiPAP?
For almost all PALS, a big part of "optimal respiratory support" is a machine called a BiPAP. "Bi" means two and "PAP" stands for positive airway pressure, meaning that the BiPAP pushes air into the body. The pressure is "positive" because air is going into the body, not being sucked out with negative pressure such as when you dehydrate food.
A BiPAP delivers two different pressures for every breath. The air pressure delivered by the machine when you breathe in (inspiratory positive air pressure or IPAP) is higher than the pressure you breathe out against (expiratory positive air pressure or EPAP).
Important myths
Some online ALS guides unfortunately repeat four major myths:
"Ideally, you will get a bilevel before you experience breathing difficulties so you can have time to get used to it."
If you want reimbursement, you will need to wait until you meet your country's thresholds. However, if you can afford it, by all means, start with a plug-in BiPAP for cash, with a script written by any clinician including your PCP — not portable, but effective.
"You will not become dependent on it, but you will probably want to use it because you may feel better when you do."
Of course, you will become dependent on it. It reduces the energy it takes to breathe. Gradually, you will rely on it for more hours as ALS progresses. This is normal. This sentence is based on a fundamental misunderstanding of these machines, as per the next myth:
"Bilevel machines do not have internal batteries, so they must be plugged in." This distinction is false. A "ventilator" like a Luisa, Breas, Astral or Trilogy also functions as a BiPAP. It's just one with a rechargeable battery. Yes, the portables may have a few more settings, but a good "plug-in" BiPAP with a backup rate and volume control can take you all the way through ALS.
"Your neurologist will prescribe the right pressure settings for you, and a respiratory therapist from a durable medical equipment (DME) company will visit to set up the machine and train you and your caregiver(s) how to use it."
Dangerously wrong! Very often, the initial settings are too high (occasionally too low) and by the nature of the controls, are not personalized to the PALS.
Seriously, don't you set your new TV or phone to your liking? If Amazon doesn't know your media preferences, how can someone miles away have adjusted your BiPAP "right" on the first try?
Respiratory therapists and pulmonologists seem to forget that you can't stuff air into weak muscles and expect magic to happen. It's like making a basket out of toilet paper.
Even the "right" settings have to be changed as you progress, and most likely your CALS will need to do that. I'm here to help. There's no set and forget here. Most deaths in ALS are because of breathing, and BiPAP is what supports that, so you do the math.
Please don't be one of the people whose settings someone has finally asked about — too late — who dies within days, having suffered for months or years unnecessarily and possibly died prematurely. it's heartbreaking.
When is it time for BiPAP?
When ALS first affects a PALS’ breathing, it is time for BiPAP, even if s/he is already on CPAP for sleep apnea.
BiPAP (“Bi” referring to these two pressures) machines allow for a higher pressure when breathing in and a lower one breathing out, to work the muscles that control breathing less hard.
If oxygenation is an issue, though that is usually not the core problem in ALS (instead, it’s too much CO2 remaining in the body because you can’t breathe it out), an oximeter can be connected to some of the newer BiPAPs and vents.
You can also check the oxygen level any time with a finger clip oximeter such as is used at doctor’s offices; these are fairly cheap and can be purchased online. Until the end of life, if then, supplemental oxygen is usually not used in ALS because more oxygen means more CO2 to breathe out. However, in short-term pneumonia, for example, it may be used. Or for some PALS, living at altitude requires it.
Often overlooked is the fact that beginning BiPAP can actually cause airway obstructions, as weak nose and throat muscles may be overstimulated by increased air flow. However, adjusting the machine’s settings and timing of breaths can preserve the benefits of BiPAP, and this is part of why the initial settings often need adjustment.
The risk of obstructive events with BiPAP is not a reason to use supplemental oxygen, which, by increasing the percentage of oxygen inhaled, generally worsens the buildup of CO2. (Some PALS do need O2 in high altitude and for other reasons).
Instead, it is a reason to get the right machine and to use it for all it's worth, adjusting settings and masks as needed.
Being on a BiPAP is different from having a tracheostomy. You decide when you want to use the machine, and if/when you no longer do. You're not permanently connected to it (even a tracheostomy can be disconnected), but if you need it and don't use it, your life will be shorter and less comfortable.
CPAP: Dangerous for PALS
In contrast to a BiPAP, a CPAP machine delivers a "continuous" pressure, meaning that the air pressure that someone inhales is within [at most, depending on the machine model] 3cm of the pressure delivered when they breathe out. This is close enough that the pressure level is considered "continuous."
You may have a CPAP for sleep apnea or know someone who does. Without ALS, in most sleep apnea, a higher exhalation pressure (EPAP) helps keep the airway from collapsing, and, because muscle tone and strength is normal, breathing out against a strong airflow is not as much an issue as in ALS.
For PALS, though, when it is too hard to exhale waste products, they will build up earlier in the disease. And there is no way that you can exhale effectively, with weak muscles, if you are trying to breathe out against a strong stream of air. Imagine how much harder it would be to blow up a balloon if it were streaming air into your nose at the same time.
So, even if you have been using a CPAP for sleep apnea before ALS, once you are diagnosed with ALS, it is essential to get a BiPAP instead, to keep your muscles from working unnecessarily and burning out prematurely, even if your PFTs are normal.
If your doctor or clinic does not understand this, and says things like, "We don't have to worry about that yet," find another immediately. Also, every BiPAP can be set up with as high an EPAP as needed, so the settings can take both pre-existing sleep apnea and ALS into account. A CPAP cannot.
Qualifying for BiPAP Reimbursement
In the US, an FVC value of <50% of predicted (in other words, you score less than half of the expected value) can be used to justify BiPAP reimbursement. Or a MIP value of <60 cm can be used.
What we do know is, in starting BiPAP, the sooner the better. Research suggests considering this, if you're financially able, when FVC declines to 80% or so, especially if it's harder to breathe lying down or your FVC or SVC has dropped significantly in the last few months.
Also, since sleep studies are now easier and cheaper (some can be done at home with a single finger or neck sensor), they are sometimes used to show that O2 is too low, CO2 is too high, and/or there is sleep apnea, any of which may justify BiPAP even if other criteria are not met, with a well-written justification from the doctor.
In Europe, the neurology federation guidelines are more liberal, and recommend initiating NIV when at least one respiratory clinical symptom or one of the following criteria is present: FVC < 80%, significant O2 drops when sleeping, or pCO2 > 45 mmHg (morning blood-gas measurements).
NICE guidelines, used in the United Kingdom (UK) are more detailed, and include a provision for men and women with orthopnea (difficulty breathing when lying down), notably that SNIP or MIP should be less than 65 cm for men, 40 cm for women, or a drop of at least 10 cm over the last three months.
Getting a BiPAP Without a DME
Some people in the US and in other countries end up paying cash for their first (and in some cases, only) BiPAP. They may not have test results that justify insurance reimbursement, have difficulty accessing equipment through the established systems or government, or face a shortage of equipment, especially given Respironics' recalls of many products, in some cases, twice, in recent years.
You may already have a CPAP for sleep apnea. As soon as you are diagnosed with ALS, trade it in or donate it to someone in need. PALS need more of a difference between inhalation pressure (IPAP) and exhalation pressure (EPAP) than any CPAP can provide (3cm at most).
You still need a prescription, generally, to get a machine, but any MD and many other clinicians (e.g., dentist, naturopath, osteopath, nurse practitioner, physician's assistant) can write one, in many countries, including the US. Make sure the prescription mentions masks and supplies, to allow you to order what you need later; specific professionals who can prescribe a BiPAP and the format of a prescription is here).
What is written for settings does not matter, since they can be changed as soon as you get the machine. It is like getting a watch that is stopped at a certain time. You set the correct time when you get the watch.
If they ship to your country or one where you have someone who can ship to you, I always recommend SecondWind CPAP. In many countries, the distributor or manufacturer (ResMed is preferred, but in some countries, Respironics or some other brand may be the only option) can sell or arrange sales to consumers.
Depending on your country and resources, you may be best served with a standard BiPAP that runs on mains power, rather than a "ventilator" like the Astral or Trilogy, which are usually more expensive (by thousands of dollars). If necessary, you may be able to buy an external battery to allow for "standard BiPAP" use outside the home, though of course this is more cumbersome than having an internal battery.
Outside the US, the Astral, Breas, Luisa, Resvent, Trilogy, or other companies' machines may be available through local distributors or a third-party supplier that ships internationally, such as SecondWind. For those in countries where import duties or inspections of medical equipment are an issue, it may be more practical to provide a shipping address for someone you trust, who can convey the machine to you.
You or your family can sell a machine that you no longer need back to SecondWind. Or donate it to someone who needs it.
Monitoring Respiration
Though American and European guidelines recommend that PALS should have pulmonary testing performed regularly, clinics may limit such tests once the numbers begin to decline and have been submitted as justification for equipment reimbursement, because they see no point in collecting further data. There is truth in this, given that new machines or settings are not recommended simply because of PFT numbers.
However, a pulse oximeter, which can be purchased from a drug store, or is part of the functionality of a smartwatch or Fitbit, may show O2 drops, an increased respiratory rate, high oxygen variability during sleep, and irregular pulse or breathing patterns, such that suggest a need to adjust BiPAP settings and/or approaching death.
If you do not currently have or want a smart watch, a Fitbit or similar wearable is a relatively inexpensive ($99 for an Inspire 3 Fitbit at this writing) to gain a rough idea of how your O2 and heart rate vary during sleep, by wearing the device for a couple of weeks to get a baseline and at least periodically thereafter.
In particular, looking at the sleeping heart rate and the percentage of sleep at which you are below it, the average O2 saturation level, and the extent to which oxygen variation jumps above the “low” threshold could give you an idea as to whether there could be something else to tweak in the settings.
You can also look at how much time you are spending in deep (stages 3 and 4) and REM sleep (stage 5) as validation of your settings. And, of course, seeking the number and timing of awakenings confirms how often a PALS is waking up long enough to need care, providing a way to test the effectiveness of adjustments to the bed, environment, or position that relate to pain and positioning as well.
Both Android and iOS phones also can be used with heart rate apps that make use of the phone camera. You can take your pulse on demand, and sometimes get other metrics as well. How well the PALS feels, breathes, communicates, receives nutrition, and sleeps is the ultimate guide for starting, adjusting, and monitoring BiPAP settings. NO numbers are a substitute. With an effectively managed BiPAP, secretion control, and means of coughing, PALS may live for years, with an FVC in the 20s or 30s, without a tracheostomy.
Do Deteriorating PFTs While on BiPAP Predict Death?
Because of learning effects (you get better at the tests the more you do them), improved BiPAP since many studies were done, and differences between machines and care across different countries, the answer is, not really.
Recently, stimulation of the phrenic nerve (in the diaphragm, the nerve that allows your lungs to expand) to measure compound muscle action potential (CMAP) has shown promise as another way of determining respiratory status. If your clinic or hospital offers this test, it's worth taking, because it avoids some of the possible biases of the classic PFT set as described above.
Tracheostomy
When BiPAP no longer works well, some PALS (<10% in the US) opt for a tracheostomy. This procedure creates an opening in the throat, into which a tube is placed, and then attached to a ventilator like the Astral or Trilogy, to provide (usually) 24/7 air at a prescribed rate. The combination of the trach and the machine is called "invasive ventilation," vs. using a BiPAP with a mask, which is "noninvasive ventilation" or NIV.
If the PALS was able to speak before the trach, a special valve can be placed to preserve speech. Likewise, if a PALS is still able to eat and drink, they can do so, following certain rules, with a trach.
A trach does change life in several different ways.
The trach tube must be suctioned to remove secretions from the throat and below, often many times a day, and a caregiver in case suction or other help is needed, must be available within a very short time frame, 24/7 limiting CALS' options if they are the primary caregiver.
With a trach, constipation can be more of a problem because you can't hold your breath, so it can be harder to push so you can poop. And because you are mainly breathing through your neck instead of your mouth and nose, your sense of smell may be reduced.
Some PALS have simply found the trach itself and/or the suctioning process too uncomfortable to want to go on. And the trach, of course, does not slow down other aspects of ALS. Research suggests that life expectancy post-trach is typically only a few years, for many reasons.
Still, it can be a good option for some PALS and if it is considered, like any other surgery, gathering several viewpoints and seeing one in person is always advised.
Diaphragm Pacing
Electrical diaphragm stimulation (diaphragm pacing system, DPS) was supposed to "train" diaphragm muscles, thereby delaying atrophy of the diaphragm, as well as the need of NIV, and therefore prolong survival in ALS. Without good data, the procedure was approved by the US Food and Drug Administration due to a shortage of options in ALS. DPS was performed on many PALS in the US and in Europe and was often used with a BiPAP.
However, beginning in 2015, studies showed that DPS actually was associated with shorter survival. In addition, more than half those who had the procedure suffered from serious procedure-related complications. Therefore, DPS is no longer considered a valid option in ALS. At some point, as with other surgeries, the procedure may become safer and more effective.
Feeding Tube Placement with Respiratory Impairment
Feeding tube or "PEG" insertion (which studies now suggest is best done in Radiology as a "RIG" for most PALS even though it's still called a PEG generically) had formerly been regarded risky in PALS with FVC < 50%.
However, studies over a decade have shown that PEG placement can be safely performed, even in PALS with severe respiratory insufficiency. Some modern masks feature openings for gastrostomy devices and allow PEG insertion under NIV. Therefore, intubation, which carries the risk that the PALS cannot be extubated afterwards, can be avoided.
One study showed that PEG was safe in high-risk PALS who underwent insertion with reduced sedation and using their BiPAP during the procedure, in a semi-reclined position. Using a Cough Assist just before the procedure if someone has been using it already, has also been reported beneficial.
So for PALS with low FVC who need a tube, if at all possible, BiPAP should be started first, and PEG should be inserted later, after stabilization of the respiratory situation under BiPAP.
BiPAP Basics
BiPAPs that are used only at home can be AC powered (plugged in), though most can work with an external battery that might cost around $400. Many PALS have one of these as a backup should their portable machine fail. But most ultimately also need a machine to go out with.
In decades past, the two most common portable BiPAPs prescribed for PALS are the Trilogy (Respironics) and Astral (ResMed). Now, since Trilogies can't be marketed due to safety concerns (as of 2024), new models such as the Breas Vivo and Luisa have entered the US.
Each of these has rechargeable batteries, allowing them to be used without a power cord for up to several hours, and can be placed in a bag on the wheelchair.
The ideal mode and settings will definitely change as ALS progresses, and can be different depending on whether someone is asleep or awake; has a cold/congestion or not; etc.
For instance, when someone is choking or coughing uncontrollably, shutting off the backup rate may be essential. There is no single lifelong recipe for any particular machine. Thus, you should know how to make necessary changes; you can PM me or post to find how/why for your/your PALS' particular machine.
If you are interested, you can monitor your own data, either through Respironics’ or ResMed’s own software, or through a free open-source app, such as Oscar (the only app that works with Mac, Win, Linux, though it only works with certain models).
Changing BiPAP settings: Why & How
This document does not include all the resources and variables needed to calculate BiPAP Vt or Va (the volume of air to set initially). When your new machine is delivered, the settings may be too high or low, or just right, depending on individual factors that are beyond the scope of this page.
To ensure that any settings adjustments reflect your height, weight, age, and medical history, if you are looking to select, purchase, adjust or better leverage a BiPAP machine or mask, please contact me via reddit (brandywinerain) or alsforums.com (lgelb), or use the contact form here.
You can’t use the same oven settings for an apple pie and a whole chicken. You can't drive at the same speed on your driveway and the freeway.
In fact, the American Academy of Neurology's ALS treatment guidelines explicitly recommend "ongoing adjustment" of pressures, and for good reason.
So, you certainly can’t "set and forget" the machine that someone depends on to keep breathing comfortably and efficiently in ALS. A respiratory therapist, even if s/he looks at the machine data, is driving using the rear view. The only way to keep the settings ahead of the disease on an ongoing basis, is by looking at the person’s response to settings in the real world, from reasonable test settings, briefly described below.
A traditional BiPAP mode is programmed to increase air flow (in Auto mode) only in the case of sleep-disordered breathing (reduced or no air flow), not any particular tidal volume.
Therefore, most often recommended for PALS is a BiPAP that includes a backup rate (PC, T, ST, AVAPS or iVAPS modes) and “volume control” or “volume support” (iVAPS or AVAPS mode option) that adjusts the pressure according to how much air the patient is moving on her own.
For portable machines such as the Astral, Breas Vivo, Luisa, and (last resort) Trilogy, the mode may be called PSV (supports pressure and volume without overriding the PALS' breathing). Except in rare cases, you do NOT want to use PCV, which does.
I have heard changing one's own settings referred to "playing with fire." Whoa! Let's unpack that.
PALS are actually "playing with fire" if they blindly accept the prescribed settings, usually generated using an online calculator for inhalation pressure ranges. These Vt or Va calculators use three variables to calculate these initial settings: patient height, a completely arbitrary choice of mL/kg volume (kind of a small/medium/large portion size choice) and respiratory rate (desired breaths per minute) as the three terms in the equation.
These calculators lack adjustments for which muscles are weak/which muscles are bearing the load instead, and other medical conditions, and have prematurely compromised the respiratory function of more PALS than I can count just from my own inbox.
The "learn targets" process some machines offer is pointless in the ALS setting, where upright vs. supine position, how recently the PALS has eaten, and other variables make a big difference. That would be like setting cruise control based on the last time you pulled into your driveway.
There are more variables in titration than the initial settings, which is as far as most RTs, pulmos, and neurologists go. In fairness, there's a shortage of all 3 and they get paid very little if at all from payors for follow-ups. The money is in new setups.
In ALS, there is no relationship among PFT results, time since dx, site of onset, age, gender and optimal BiPAP settings. The only low-risk means of empiric titration is "start low," often way lower than the rx that are written for PALS and entered into the machines they are delivered.
Moreover, most PALS are never re-evaluated to verify if they have the right synchronization between their natural breathing and the machine (Ti, trigger, cycle, AVAPS rate/rise time, other settings).
I see only lower risk in a lower-setting baseline with personalized titration and synch/comfort settings. And though I've never asked for testimonials, the PALS I've helped are testaments to that.
What About Pre-Existing Conditions?
If you have pre-existing lung problems and especially a history of smoking, your PFTs may have been abnormal even before ALS (whether you knew or not), and so these conditions should be reflected in your BiPAP settings. Conditions that affect breathing in ALS include previous spine surgery, asthma, COPD, and more.
Likewise, if you had apnea and used a CPAP before ALS, the BiPAP settings should be adjusted accordingly (e.g., you may need a minimum EPAP that is higher than the machine minimum often advised in ALS). Please send me a message or post if you need help.
Some Basics on Settings
For simplicity, since most PALS have a Trilogy at this point, those settings are used below, though some models differ from others so not all will apply. If you have an Astral or other ResMed machine (better than Respironics!), or a Breas or Luisa or some other machine, let me know.
First, ensure access to data and settings. If there is a closed lock on the screen, you will first need to enable “Detailed View” of the machine data, which helps us see how things are working, and “Full Access” to the settings so you can adjust them as needed.
To do this, hold down the alarm button and down arrow together until you hear a beep. You have unlocked the machine. A few machines require a different combination; just let me know if that doesn’t work.
Now, on the screen, select Settings and Alarms. Then select Detailed View and Full Menu Access by scrolling through the menus and saving those settings. You will always confirm your changes, so you cannot mess anything up. If you make a mistake, exit without saving.
So after you’ve taken the machine off cruise control, from then on, you just do the alarm/down arrow thing any time to modify other settings. ALS is a progressive disease, so it’s common to change settings as you go.
Many people disable all the optional alarms, if they are not already. The really important ones, like a power failure, can’t be turned off.
Below are the settings I would recommend as a starting point, with notes. Some require an individualized recommendation, as noted, or are slightly different depending on machine model – just ask. This is the correct order for changes, as some settings are contingent on others.
Mode: Depending on the machine, the best mode to start with may be called S/T or PSV (TgA) or AVAPS or AVAPS ST. Some physicians specify PC or AC mode. But it is better to use the PALS’ own length of breath for many reasons, including comfort and not having to "fight" the rhythm of the machine.
AVAPS ON: This is the setting that tells the machine to change the pressure based on the volume of air being moved, not just to keep it the same all the time. This is very important as someone goes from light to heavier sleep and back.
There may be an AVAPS-AE option. Most PALS with previously normal breathing don’t need it, so that can be OFF. If you have asthma, COPD, or sleep apnea before ALS, ask me.
AVAPS rate: With someone new to the machine, I would start with 1, which is more gradual. This setting controls how quickly the machine adjusts to air volume needs. The higher it’s set, the more often pressure changes will likely result. Many people end up 3-4, in the middle. But if you set it at 1 and the PALS feels like the machine is not adjusting quickly enough, you can take it up then.
Target volume/Vt (Va for ResMed machines and calculated differently): For ~90% of the PALS who send me initial settings, they are too high, often due to misunderstanding of what weak muscles really mean. You can’t wake them up with high air flow.
To look at it another way, do we tie people who can no longer walk to the nearest pillar so they can stand? No, because of dead motor neurons and associated atrophy, their muscles and other tissues are no longer able to support their weight.
Similarly, once the muscles that support the work of breathing have begun to wither, in most cases they can no longer support the breathing patterns that used to be autonomic and not even noticed on a conscious level.
This is the volume of air that the machine will use pressure changes to maintain, so it’s very important. To get a starting point, contact me on the Forums and I'll ask where it’s currently set, what the PALS feels, height, weight, age, and lung disease/smoking history.
For the few who may need to increase that setting, I always advise increases of no more than 10% at a time.
Target volume will also change as the disease progresses; some people go up and others down, depending on which muscles are weak. This is the setting that most determines both comfort and value of therapy, so give changes a chance before drawing conclusions.
Maximum IPAP (IPAP max): we will base this on the tidal volume. Most often it will be 10-18. Often it has been set way higher, overstuffing the lungs. In some PALS, a high IPAP also irritates the vocal cords and/or throat, causing coughing, choking, and/or speech impairment. Look at it this way -- when you’re in a windstorm, do you speed up your driving to the speed limit or beyond, or do you weave your way mindfully through the wind and traffic to ensure your car and the wind co-exist?
Minimum IPAP (IPAP min): There must always be at least a 4 cm/Hg difference between EPAP and IPAP, the minimum for PALS. So we will set IPAP min based on the IPAP max.PS min/max -- this is the minimum and maximum difference between IPAP and EPAP. We want this to be at least 4 and most PALS do best when it's at least 6. The max will be based on how high we want IPAP to go, which depends on Vt/Va but also on the mask, bulbar weakness, and leak.
EPAP (exhalation pressure) – Almost always, absent pre-existing lung disease, 3 or 4, whichever is the lowest available, is appropriate. On some ResMed and other machines, it is 2.
We want to keep this low unless someone has pre-existing obstructive apnea or COPD that could cause their airway to collapse at low expiratory pressures. This is the pressure that the PALS is breathing against to exhale.
BPM/backup rate/RR/breath rate. The machine will only start breaths on its own if the backup rate is not being achieved through her own breathing. I usually recommend 8 since some PALS breathe slowly in deep sleep. Most PALS never trigger the backup rate if other settings are set appropriately and if the flow volume, speed and interval are just right.
Inspiratory time (Ti) – this only applies if the backup rate kicks in (meaning the person is not triggering their own breaths often enough -- this can often be fixed by changing trigger sensitivity and other settings, but not always). For most PALS without pre-existing lung problems, I recommend 1.3 (seconds) so those breaths are not too short. "Ti min" should be
0.5 seconds to allow for shallow breathing when needed.
Trigger type -- “Flow” is usually appropriate. Rarely, there will be pulses during sleep, and you will switch it to Auto Trak. The flow trigger selection enables you to set sensitivity manually, instead of having the machine do so.
Trigger sensitivity set to 3 [then if the PALS wants to breathe more often, decrease it or vice versa]. This controls how sensitive the machine is to the PALS beginning to inhale. On a ResMed machine, this will be between "Very Low" to "Very High."
Using S/T mode, after you change trigger type to flow trigger, you will get access to the flow cycle sensitivity setting, which I would start at 20% [then if the PALS thinks breaths are too long, increase it or vice versa]. This controls how sensitive the machine is to the PALS’ beginning to exhale and keeps them from getting “stuck.”
Special circumstances: coughing, choking, mouthpiece breathing weird noises
• When coughing, turn off backup rate and Vt, set low fixed IPAP/EPAP, e.g. 10/4.
• When using a mouthpiece, same as for coughing, and turn EPAP OFF since you are not exhaling into the mouthpiece
• If swallowing a lot of air, try reducing IPAP max first, not Vt
• If weird noises, for Respironics machines, change flow trigger to Auto Trak first, then if that doesn’t work, reduce rise time and/or flow trigger sensitivity (if you went back to flow trigger)
Mask notes – Some people are prone to panic attacks when the mask is put on, and others continue anxious while wearing a mask. In some cases, this concern can be treated with small amounts of opioids or benzodiazepines. In others, this represents ongoing depression and/or anxiety that can be addressed with a once-daily antidepressant.
However, in ALS, when BiPAP is set properly, extreme anxiety during use is relatively uncommon because PALS who have experienced air hunger and breathlessness find more comfort in BiPAP. Thus, if anxiety and discomfort using the mask prevent its use, it may be time to consider adjusting the settings, not giving up.
Many PALS get used to the mask by wearing it during the day while watching a movie, listening to a book, having conversations, or doing routine tasks.
You want the mask to be held on by suction, not force — kind of like sitting in a bean bag chair or floating in a pool. So it’s usually best to set the length of the straps while the air is on, letting the suction guide the mask position. Not too tight, not too loose.
Leak should be less than 25 on a “regular BiPAP,” but may go up to 50 or so on a portable machine. It’s really not the number that’s important, as much as whether it’s preventing someone from relaxing/sleeping, and/or whether compensating for leak is increasing pressures to an uncomfortable level.
Since opioids and benzodiazepines may reduce PALS' respiratory drive, they should be used with care. On the other hand, at small doses, they may improve the ability to use BiPAP at all. In later disease, they may also reduce air hunger.
However, PALS who are heavily medicated for anxiety, depression, and/or pain, often with drugs that depress the central nervous system, may control secretions less well, which could increase choking risk. They may also have a drier nose/mouth, which could increase the risk of nose bleeds, cracked lips/tongue, and difficulty with BiPAP.
If this sounds like a Catch-22, there is a way out. More attention to positioning and doing something interesting can reduce or eliminate the need for mood-altering meds, while making it more pleasant to think about waking up in the morning.
The Biotene or grapefruit seed extract products and increasing hydration, as well as adjusting the machine humidifier and climate control settings, may help with dryness. For those PALS for whom it is safe, a sugar-free cough drop such as Luden's can be useful.
Nasal masks may be less heavy and are easier to speak with, if that is still possible. However, sores can develop on the nose bridge. Paper tape and/or fabric mask liners may prevent or help heal these, along with ointment such as grapeseed oil extract.
It is untrue that people with deviated septa or mouth breathers can't use nasal masks. A lot of mouth breathers can convert (without chin straps or mouth tape that hinder natural breathing) as breathing becomes more restrictive than obstructive — in other words, as it becomes more of a chest muscle than airway problem. And since one of the prime directives in BiPAP is not providing more air than someone can handle, that obviously includes the nose. I see far too many PALS suffering unnecessarily in full face masks, especially during the day. Give multiple nasal masks a fair trial first.
With bulbar weakness, especially if onset was bulbar, air might escape due to incomplete closure of the mouth, especially at night during sleep. For this reason, some PALS use a full-face mask at night and a nasal mask during the day.
Mask options have expanded considerably; a full assortment can be seen (and generally ordered with return options) at sites such as cpap.com. As an example of a newer silicone mask, the AirFit full face mask is a lighter version than some, and some PALS may find the air flow at the top of the head more comfortable. A nasal AirFit version is also available.
As an example of a nylon mask, resembling a parachute, some PALS prefer the Circadiance line.
Mouthpiece Ventilation
For PALS who can still "latch on" to a mouthpiece, daytime mouthpiece ventilation can be used when continuous machine support is not needed. This involves using a plastic mouthpiece attached to the hose (which is mounted vertically within reach), instead of a mask. When the PALS needs a breath, they suck in on the mouthpiece.
With this option, you turn off the backup rate, trigger, cycle, and Ti min/max settings completely.
Mouthpieces are available online for a few dollars. They are the same as used for nebulizers – 15mm internal dimension (ID) and 22 mm outside dimension (OD). Depending on the PALS, some may do better with an angled one, others straight. Since they're so cheap, it may be worth trying both.