Managing Secretions

The first step to managing saliva, mucus, and "gunk" is paying a lot of attention to the foundations of avoiding other diseases that affect breathing, and breathing in clean room or outdoor air.

Why Are Secretions a Concern?
PALS produce a normal amount of saliva, but they can't easily swallow it, move it around, or cough it out as healthy people do. Likewise, they can't readily swallow, sneeze, or cough out mucus. And of course, sometimes the two are mixed.

So they may drool, get choked up, need to cough but be unable to, and since their breathing is already weak, secretions can make it worse in a hurry. Aspiration of fluids into the lungs can cause pneumonia and even death.

Thus, managing secretions (saliva and/or mucus) that get "stuck" or result in drooling, coughing, and choking is key in ALS. If secretions build up, and the PALS becomes ill with a cold, flu, COVID, or RSV, they may also get pneumonia and other severe complications more easily. And those complications may be more difficult to treat.

Viral Transmission & Air Quality
Secretions are obviously a greater risk with any respiratory illness. PALS have died from a "simple cold." So, one of the most life-extending things that P/CALS can do is to avoid all the contagious respiratory illnesses (RSV, flu, pneumonia, colds, Covid), by:
◦ Requiring visitors to mask up and be vaccinated (vaccinations reduce severity, which reduces the risk of transmission),
◦ Getting all the vaccines and boosters recommended for high-risk people (including the new Covid and RSV vaccines available). Some reputable scientists recommend that vulnerable people like PALS get vaxxed for Covid every 6 months.
◦ Running air purifiers like a Corsi-Rosenthal box or any fan with a HEPA filter, being careful to avoid ozone-producing purifiers. AirFanta is a commercial implementation of the latest science.
◦ Masking up in all indoor public space, even if no one else is there (virus lingers in the air)
◦ Using an Aranet or other CO₂ monitor to assess risk
◦ Avoiding crowds as much as possible, even outdoors
◦ Running the furnace fan continuously if no one else is sick
◦ Opening windows when weather allows (especially when visitors are present), etc.
◦ Visitors from out of town, ideally, should quarantine for a few days and self-test before seeing the PALS. The pandemic is not going anywhere, and there is no such thing as "mild COVID."

All PALS and CALS, along with other family, regular visitors and caregivers, should have all recommended vaccinations, since all PALS are high-risk for complications.

◦ CALS should be careful about potential irritants that could trigger coughing or worsen breathing, including essential oils, incense, scented cleaning products, perfumes, cooking or tobacco smoke, vaping, etc.
◦ Unscented CPAP mask-specific wipes are recommended for mask cleaning. Other commercial cleaning wipes should never be used. Soap and vigorous rubbing break down the mask cushion, and many cleaners break down the silicone against the skin and/or irritate the nose or mouth.
◦ To avoid irritation, leaks and infection, while keeping comfortable as possible, good habits include replacing furnace and BiPAP filters on schedule, rinsing out hoses; cleaning mask cushions every day and the shell as needed (making sure no holes are blocked); washing the PALS' face before applying the mask; using only distilled water in the machine humidifier; using heated tubing if your machine supports it.

Prevention First
It is always easier to prevent secretions than get them out. Though hydration and adequate humidity (but not too much) are top of the list, varying the PALS' position in bed, chairs and any other positioning surface helps, as does keeping the head up and extending the upper torso away from the jaw (in the wheelchair and bed, this is the equivalent of tilt + recline). At night, as sleep becomes deeper, the bed may require adjustment.

For some people, selected essential oils in a diffuser may improve secretions. For others, they make them worse. Consideration of allergies is advised.

Saliva that cannot be managed with assisted coughing, suction, paper towels, etc.: Many PALS use pineapple or papaya juice or chewable papaya tablets, which also benefit digestion. Bromelain tablets are also an option. Warm liquids (including lemonade, apple juice, coffee, and tea), sodas and Alka-Seltzer are all worth trying, if there are no medical contraindications.

Avoiding cow-based dairy is another prevention tactic that helps some. Oatmilk and nut milk-based products can substitute, but read labels to ensure you compensate elsewhere for the nutritional differences, if needed.

Cough syrups that contain guaifenesin (it is also available by itself in liquid form on Amazon) are often the first logical OTC step. But skip the ones with decongestants, unless someone has an active respiratory illness and no contraindications, since they affect the heart, take energy to metabolize, and can interact with other medications.

If stronger measures are needed, the EFNS guidelines recommend amitriptyline, oral or transdermal hyoscine, or sublingual atropine drops, while the NICE guidelines additionally mention glycopyrrolate, especially for PALS with cognitive impairment, because of fewer central nervous system (CNS) side effects than the other meds.

Injection of botulinum toxin B ("Botox") in the parotid and submandibular glands has been shown to improve drooling/choking in about 80% of PALS and is usually recommended when PALS do not respond enough to the drugs listed above.

Some doctors consider radiation therapy of salivary glands in PALS who do not respond sufficiently to medications.

Mucus: Though many of the same approaches to saliva also keep mucus from building up too often, some PALS with weakened cough suffer jelly-like bronchial secretions that may lead to panic attacks and suffocation sensations.

PALS use humidification of room and machine air (most people should be using a humidifier or HME with their BiPAP anyway) to reduce or dilute the mucus. Options for thicker secretions include nebulizers with saline, anticholinergic bronchodilators often used for asthma, or furosemide (a diuretic, not recommended unless your PCP says it's OK and you don't mind peeing a lot).

Other treatment options include "mucolytics" like N-acetylcysteine (NAC), aka Mucomyst, and beta blockers like propranolol, the latter subject to heart and lung conditions.

Suction, Assisted Coughs, and "the Vest"

Some PALS benefit a lot from suction machines. Try to use only olive tip attachments for safety. Your DME can provide them or you can buy them on line.These can be used (always with care!) in the nostrils and/or back of the mouth.

Some DMEs provide a suction machine that doesn't "suck enough." DeVilbiss is a top brand. The EasyVac Go that some DMEs supply delivers 483mmHg of suction, but you are looking for a higher number, preferably in the 600s. Suction machines don't need to be small, cute, or sleek. You're using them at home or while traveling, not in a Good Housekeeping spread.

You can buy a suction machine for cash or rent via Medicare.

Unfortunately, drugs like those above may do more harm than good for PALS who cannot effectively eliminate secretions, because drying them out can make them harder to remove. Therefore, many clinicians believe that mucolytics should only be used in combination with assisted cough devices.

Some PALS cannot process mucus without the Cough Assist, an expensive piece of equipment that a doctor orders and a DME delivers. The Cough Assist helps the PALS' cough be more productive. However, for PALS with other lung conditions or connective tissue disorders, it could be dangerous.

Others have enough muscle tone (it's not necessarily overall tone, but which muscles/fibers are weaker than others) remaining to basically allow mucus to go back into the GI tract, whether always or at certain times, as you would if you didn't have ALS.

A baby bulb aspirator, available in drug stores, may help keep nasal mucus from dripping. If you have a suction machine, you can carefully use an olive tip attachment to suck up secretions in the nose or at the back of the mouth.

Well-hydrated PALS will often have thinner mucus that "goes back down" more easily. Diet by mouth or which formula/blends you use in a feeding tube can also affect the amount/consistency/irritation factor.

Because it's an invasive process, some PALS find that the CA is more irritating than helpful, again, either always or at certain times, and adjust their use of it accordingly. It is worth experimenting to see what works best.

There may also be a role for "the Vest" [airway clearance system/oscillating vest] here, which is used to loosen secretions before they are coughed up with or without mechanical assistance. However, these vests, one made by Baxter and one by Hill-Rom, are expensive and again, not right for everyone.

PALS with bulbar weakness may not generate effective coughs even when assisted by a machine, due to collapse of the upper airways (that essentially no longer have muscle tone) during the cough. A breath-stacking technique using a lung-volume recruitment bag may be an equally effective low-cost alternative. Or the two may be combined, with or without manual cough support from a CALS.

PALS with sticky, thick secretions may get more effectiveness and comfort from BiPAP if they use assisted cough to free the upper airways before starting the BiPAP. Of course, if BiPAP is being used continuously, this cannot happen.

BiPAP settings also play a greater role in secretion management than you might think; especially if pressures are too high, that can add to secretions. "Just right" pressures can help them move along without irritating the airway in a way that triggers coughs.

Secretions can build up in a hurry when position changes or the work of breathing increases dramatically, and that, in turn, can worsen any airway collapse. So if someone is having breathing difficulties on BiPAP during transfers or toileting in particular, it may be necessary to change their settings during these high-energy-expenditure times — increase the backup rate, remove Ti min/max, and watch/ask about indications that their pressure/volume settings should be adjusted during this time as well.

Benzos, Morphine & Supplemental Oxygen
Benzodiazepines like Valium, Ativan, and their cousins make breathing more shallow (restrict the depth of each breath because they affect the central nervous system that drives breathing), so it’s harder for a PALS on or off BiPAP to get air in and help get it out.

In turn, secretions accumulate when less air is being moved. Remember, what keeps a healthy person from needing suction is that you are moving air — it's not that aPALS is making more mucus.

Often you will see in the BiPAP monitoring screen, or just hear, that someone moves less air in this scenario. Then they feel hungry for air and often a clinic or hospice will advise benzos for anxiety, which only slow down air and secretion clearance more. It's a vicious cycle, because someone who's sedated has a mechanical block — secretions — that are preventing air flow, and reduced air flow prevents the secretions from moving along as well.

At this stage, hospice or some other clinician may also suggest supplemental oxygen "for comfort." But all this does is deliver extra oxygen and therefore more CO2 gets trapped in the body more quickly, causing headaches, apathy, confusion, nausea, loss of appetite, etc. So then the PALS may have even more drugs thrown at them so they "don't suffer," when secretion management is the real key, until. the very end of life. F

or this reason, I have helped PALS who are not at the very end stop their O2, improve their BiPAP settings, and use fewer sedatives, all of which in themselves improve secretion management along with everything else listed here. That way, they can actually be in a position to consider what they want the end of life to be, and live whatever life they want as long as they can.

Positioning
As noted above, PALS who are lying down often find it more difficult to use the muscles that control breathing, even the muscles like the abdominals that come into play when bulbar muscles begin to weaken.

Therefore, wherever the PALS is, whether in bed, a sling, a chair, or a wheelchair, the tilt/recline functions (or their equivalent, or whatever is available, including pillows and arm support) should be used to keep the upper body at a comfortable angle that allows the muscles used for breathing to be as active as possible.

Often, neck muscles are weak, and that can lead to slumping that further compresses the nose and/or throat. That's why you want the neck extended so that the airway is elongated but not tight.

Because bed positioning in ALS aims for better breathing and less pain, it can be complicated. That’s one reason why you want a hospital bed that allows for “reverse Trendelenburg” positioning, not just “bed up down, head up/down, and feet up/down.”

Though more expensive (you will sometimes see these beds referred to as "ICU beds"). these beds are much more comfortable for PALS because the torso can bend at the hips as you do much of the day, and also tilt for urinal use.

In addition, trial and error will reveal the angle that best prevents secretions from being feeling "stuck" in the throat or airway leading to the nose. Just as with pre-ALS illnesses, what you feel in your nose, throat, sinuses and ears does not necessarily correlate with what secretions or pressure are necessarily there.

So, when you use positioning to reduce ALS' impact on breathing and comfort, part of it is about real forces like gravity and part is the person’s individual perception. That is why “angle the head at 15 degrees” may or may not be good advice for your PALS.

In a wheelchair, in addition to pressure relief, periodically tilting the wheelchair back can also somewhat loosen secretions and/or prevent buildup. However, ultimately the air quality and medical support, including BiPAP, matter more.