ALS Respiratory Changes

Navigating the complexities of ALS respiratory challenges can be overwhelming from symptoms to what support you will need. The below information will provide you with tailored information on symptoms, support, breathing exercises, and essential respiratory equipment, so you can empower yourself with the knowledge and support needed to maintain comfort and quality of life in the face of ALS respiratory difficulties.

ALS Respiratory Symptoms

ALS is a neurodegenerative disease that weakens voluntary muscles throughout the body. For the vast majority of people, ALS symptoms first appear in one of the limbs or in the muscles that control speaking and swallowing. Just a small percentage of people notice respiratory symptoms first.

Yet as ALS progresses, it will eventually affect the respiratory system. When ALS weakens the diaphragm (your primary breathing muscle), it becomes harder to fully inhale and exhale. This can lead to common ALS respiratory symptoms such as:

Trouble breathing while lying down
Shortness of breath
General fatigue
The need for more frequent naps
Waking with morning headaches
Feeling groggy or “out of it” in the morning
Difficulty finishing a sentence without taking a breath
A weaker cough

Whether or not you are experiencing any respiratory symptoms, it is important to attend an ALS clinic and have your breathing measured regularly. Oftentimes, breathing tests will detect a decline in breathing function before people living with ALS notice any symptoms. Getting respiratory support early can help you sleep better, have more energy, be more comfortable, live longer, and have a better quality of life.

ALS Respiratory Support

When you’re diagnosed with ALS, it is important to attend an ALS clinic or see a medical team that specializes in ALS. A respiratory therapist or nurse will monitor your breathing at each visit, suggest respiratory devices that may be helpful for you, and guide you every step of the way.

After you have received the diagnosis, your neurologist will schedule your first clinic visit. If you do not live near an ALS clinic or cannot attend one, contact your local ALS organization to ask if they can recommend a local neurologist who specializes in ALS or other medical professionals who can help.

If attending an ALS clinic is not possible, ask your primary care doctor or neurologist if they can either monitor your respiratory numbers or recommend a respiratory therapist who can do so. Once you qualify for a certain respiratory device, your doctor or neurologist will order the device through a local durable medical equipment company.

If any respiratory issues or concerns arise between visits, contact your medical team to ask questions or schedule a separate appointment.

Respiratory Equipment

Respiratory equipment is therapy for your respiratory system. It can help you sleep better, have more energy, and experience a better quality of life. Studies have shown that using respiratory equipment on a regular basis can help you live longer. Starting early can help you get used to the devices and begin feeling better sooner.

Most respiratory equipment is noninvasive, which means you can take the mask or interface on and off whenever you wish. If you get a tracheotomy at a later stage in the disease, however, you will become dependent on a mechanical ventilator to breathe for you 24/7.

Breathing Exercises for ALS

There are some daily breathing exercises that you can do early in the disease to help you open and expand your lungs, breathe more easily, and have a stronger cough.

“Breath stacking” involves taking a series of short breaths on top of each other without exhaling. After holding your breath for a few seconds, you fully exhale all at once. With expiratory and inspiratory muscle training, you use a small, handheld device to inhale and exhale against a resistance level that is set and adjusted according to your breathing test measurements. Other helpful breathing techniques include huffing and deep diaphragmatic breathing.

Ask your ALS respiratory therapist, pulmonologist, or neurologist about breathing exercises that might help you. Follow their guidance before trying these exercises on your own.

Improving Your Sleep

Getting a good night’s sleep is important for your health, well-being, and energy level. If you are finding it hard to get a solid night’s sleep, or are finding it harder to breathe when lying down, it may help to prop yourself up with pillows or a bed wedge. If you are still not getting the rest you need, it may be time to get a bilevel (BiPAP) machine or use a portable ventilator in bilevel mode to assist you with breathing while sleeping. This should improve your quality of sleep and help you feel more rested when you wake up.

Shortness of Breath

If you are experiencing shortness of breath, it is likely the result of a weakened diaphragm muscle. This makes it harder to expand the lungs enough to fully receive oxygen and fully exhale carbon dioxide. Typically, a bilevel (BiPAP) machine or a portable ventilator will help. Most people living with ALS do not need supplemental oxygen. The exception is when your oxygen levels drop below normal levels, which is usually from an underlying lung condition such as COPD or emphysema.

If you develop sudden shortness of breath, it is important to seek immediate medical attention, as the cause could be something other than ALS, such as a blood clot in the lungs.

Keep your primary doctor

Even after you connect with a team of ALS specialists, you should still continue to see your primary care doctor. If you get the flu, a cold, or an infection, it is especially important to see your doctor as soon as possible to get it cleared up.

Monitoring Your Breathing

Your medical team will begin monitoring your breathing even before you notice any respiratory symptoms. If you attend an ALS clinic, a respiratory therapist or nurse will ask about any changes you’ve experienced and perform one or more simple, noninvasive tests to measure your progression.

Forced Vital Capacity (FVC) is the primary test used to evaluate the breathing of ALS patients. It measures how quickly you can expel air from your lungs.

The Maximum Inspiratory Pressure (MIP) test measures the strength of your inhale through your mouth. The Sniff Nasal Inspiratory Pressure (SNIP) test is the same as the MIP, but it measures your inhale through your nose, which can be helpful for bulbar patients who are unable to form a good seal around the mouthpiece.

Your team might also recommend a capnography study, which measures your oxygen and carbon dioxide levels as you sleep. This can alert your team early on if your diaphragm function is impaired. This study takes place at home, with a clip on your finger and a thin tube in your nose.

The results of these tests, along with the observations you share, will give your team the information it needs to recommend the best care and respiratory equipment for you. The results from these breathing tests will also provide the documentation your neurologist will need for Medicare, Medicaid, and private insurance to cover your respiratory devices.

ALS Respiratory Devices

Your ALS clinic or medical team will monitor your breathing, provide respiratory support, and prescribe the right respiratory devices at the right time. Using respiratory devices can improve your quality of life and help you live longer.

These are the most common types of respiratory devices that help people living with ALS:

A bilevel (BiPAP) is a small breathing machine that delivers pressurized air through a face mask to assist with both inhaling and exhaling, primarily during sleep.

A portable ventilator (Trilogy) can help you breathe either noninvasively (via a mask) or invasively (via a trach). It has a bilevel mode for sleeping and daytime breathing support.

A cough assist machine helps your breathing by clearing phlegm and fluid-like secretions from your lungs. Using it daily can also help condition the diaphragm muscle, even if no secretions are present.

A suction machine is a simple device, similar to the wand at the dentist’s office, that uses suction to remove excess mucus and saliva from your mouth.

Masks and Interfaces

When your bilevel (BiPAP) machine or portable ventilator (Trilogy) is delivered, a respiratory therapist from a durable medical equipment (DME) company will bring a variety of mask options and recommend the one that will be the most appropriate for you. This may depend on how you breathe at night and how well the different masks fit. Cough assist machines will arrive with the appropriate mask included.

These are the most common types of masks and interfaces for people living with ALS:

Nasal Pillows

With just two little cushions that seal at the base of your nostrils, this is the lightest and most compact interface option. Nasal pillows are generally good for people who breathe through their nose when sleeping. Because of its low profile, you can still wear glasses, read, watch TV, talk, and eat with nasal pillows in place. If you have a lot of facial hair, nasal pillows may help you get the tightest seal. If you tend to breathe through your mouth at night, nasal pillows may not be your best option, though you can ask about attaching a chin strap that can help keep your mouth closed.

Nasal Mask/Interface

The nasal interface is larger than nasal pillows but less bulky than a full face mask. Triangular in shape, it sits on your face and covers your full nose. The nasal interface is generally good for people who breathe through their nose when sleeping. If you tend to breathe through your mouth at night, a nasal interface may not be your best option, though you can ask about attaching a chin strap that can help keep your mouth closed.

Full Face Mask

The traditional full face mask is more bulky than the other interfaces, but it does allow the option of breathing through your nose or mouth. This can be especially helpful if you tend to get congestion that makes it harder to breathe through your nose. If your air pressure settings are high, the full face mask will be the most comfortable option. This larger mask can make it hard to read, watch TV, or sleep on your side or stomach. There is also a higher chance of air leaking with a full face mask.

If your mask or interface is uncomfortable or doesn’t feel like it is fitting correctly, schedule another appointment with the respiratory therapist who set everything up. Making adjustments or trying a different mask or interface may solve the problem. It may also just take some time to get used to wearing a mask and using a new device. The benefits of using a bilevel or portable ventilator in bilevel mode are too great to give up because something doesn’t feel right at first.

Bilevel - BiPAP Sleep Therapy

Bilevel (BiPAP Sleep Therapy)

A bilevel machine, commonly referred to as a BiPAP, is a breathing machine primarily used during sleep that delivers pressurized air through a mask or interface to assist you with both inhaling and exhaling.

A bilevel (BiPAP) can help you sleep better, wake up feeling more rested, and have more energy during the day. In addition to improving your quality of life, research has shown that using a bilevel early and regularly can help you live longer.

How do bilevel machines work?

When the diaphragm muscles weaken and your body is unable to fully inhale oxygen and fully exhale carbon dioxide, your mind can begin to feel foggy and you can get morning headaches. The bilevel machine assists you in being able to take a bigger breath and exhale more fully.

The bilevel is noninvasive, which means you can take it on and off and use it whenever you wish. You will not become dependent on it, but you will probably want to use it because you may feel better when you do. Bilevel machines do not have internal batteries, so they must be plugged in.

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. The respiratory therapist will try to find a face mask or interface that lets you sleep in your normal position, whether on your stomach, back, or side.
The respiratory therapist will also show you and your caregiver how to use and refill the humidifier chamber, which humidifies the air to help prevent dryness in your mouth, nose, throat, and airway.

Can I use it during the day?

Yes. If you are having difficulty breathing during the day, or simply feel like you could use a little more breathing support, you can use your bilevel while you are awake with the same mask or interface you use when you sleep. This can give your diaphragm a break and increase your energy level. You can also use your bilevel during naps, just as you do when sleeping at night.

If you are finding that you need your bilevel more throughout the day, speak with your clinic team about options that might work better for you.

When should I get a bilevel?

Your ALS clinic team or neurologist should monitor your breathing every visit and let you know when you will qualify for and can benefit from a bilevel. Ideally, you will get a bilevel before you experience breathing difficulties so you can have time to get used to it.

How can I get one?

Your neurologist must submit your pulmonary function test results and documentation that demonstrate you need breathing support during sleep. The durable medical equipment company will process the order through your insurance, deliver the bilevel to you, and train you how to use it. Insurance companies do not usually require ALS patients to do a sleep study to qualify for a bilevel.

Using a Portable Ventilator in Bilevel Mode

Using a portable ventilator in bilevel mode provides the same therapy as a bilevel machine. Portable ventilators have more settings and uses, but they are more expensive. Your medical team will recommend the device they think is best for you based on your breathing measurements and symptom progression.

How much will I have to pay?

Bilevels are considered rentals for the first 13 months and are covered through Medicare, Medicaid, and most private insurance plans. After 13 months, the machine becomes yours and your supplies will continue to be covered by insurance.

Medicare will cover 80% of the cost of your monthly rental. Medicaid, supplemental plans, and secondary private insurance should pay for the remaining 20%. If you have Medicare but do not have Medicaid, a supplemental plan, or secondary private insurance, you will likely have to pay the remaining 20% out of your own pocket.

If you do not qualify for Medicare—but have Medicaid and/or private insurance—find out what your durable medical equipment (DME) benefits will cover. If you have a Medicare Advantage Plan, be sure to ask about your DME benefits.

If you are facing out-of-pocket expenses that you cannot afford, talk with your DME provider and/or local ALS organization about financial assistance options. Respiratory equipment is not generally available from loan closets, but you can always ask.

When your bilevel machine is delivered, a respiratory therapist will bring a variety of mask options and recommend the one that will be the most appropriate for you. This may depend on how you breathe at night and how well the different masks fit.

What if I’m having trouble using my bilevel?

Some adjustments may be needed when you first start using a bilevel. If you are having trouble, schedule another visit with your respiratory therapist. Together, you should be able to resolve the issue. The pressure may need to be adjusted, your mask may be leaking, or you might simply need more time to get used to it. Whatever the problem, the benefits you’ll receive from your bilevel are too great to give up and stop using it.

Portable Noninvasive Ventilation

If you are experiencing respiratory symptoms like shortness of breath, noninvasive ventilation (NIV) can provide helpful breathing support through a mask that you can put on and take off as needed.

Studies have shown that noninvasive ventilation can alleviate respiratory symptoms, improve quality of life, and help you live longer. It is one of the most effective interventions available for people living with ALS. You can receive noninvasive ventilation either through a bilevel (BiPAP) machine or a portable ventilator (often referred to by the brand name Trilogy).

Bilevels (BiPAPs) are smaller and less expensive than portable ventilators, but they are not portable. Bilevels are primarily prescribed for nighttime use. Portable ventilators have more settings and functions and are more equipped to support your breathing during the day as well.

Portable ventilators have internal batteries, and you can buy backup batteries if needed. This means you can unplug the device and receive breathing support while moving around your home, going out, or traveling. Portable ventilators typically weigh under 12 pounds and come with travel bags that can hook onto the backs of wheelchairs.

Your medical team will recommend the device they think is best for you based on your breathing measurements and symptom progression. If you start with a bilevel, you can switch to a portable ventilator when needed.

Receiving noninvasive ventilation (NIV) is very different from invasive ventilation, which involves creating a surgical opening in your trachea and becoming dependent on a ventilator to breathe for you 24/7. Portable ventilators can be used for both invasive and noninvasive ventilation.

How can I get a portable ventilator?

Your ALS clinic team or neurologist will monitor your breathing every visit and let you know when you qualify for a portable ventilator.

You will need to have a face-to-face meeting with your neurologist, usually during a clinic visit, to get your portable ventilator prescribed for noninvasive use. Your neurologist will need to have pulmonary function test results and documentation that the durable medical equipment (DME) company can use to process the order through insurance. A respiratory therapist from the DME company will then deliver your ventilator and train you and your caregivers how to use it.

If you are using a bilevel and it is still considered a rental, you will need to return it when the portable ventilator is delivered.

How much will I have to pay?

Portable ventilators are considered long-term rentals and are covered through Medicare, Medicaid, and most private insurance plans.

Portable ventilators are more expensive than bilevel (BiPAP) machines, so the monthly payment will likely be higher. Supplies such as masks, tubing, filters, and humidifiers, as well as periodic maintenance, will be included in your monthly rental fee.

When your portable ventilator is delivered, a respiratory therapist will bring a variety of mask options and recommend the one that will be the most appropriate for you. This may depend on how you breathe at night and how well the different masks fit.

What if I’m having trouble using my ventilator?

Some adjustments may be needed when you first start using a portable ventilator. If you are having trouble, schedule another visit with your respiratory therapist. Together, you should be able to resolve the issue. The pressures may need to be adjusted, your mask may be leaking, or you might simply need more time to get used to it. Whatever the problem, the benefits you’ll receive from your ventilator are too great to give up and stop using it.

Cough Assist Machine for ALS

Cough Assist Machine

A cough assist is an airway clearance device that helps your breathing by moving phlegm and fluid-like secretions from your lungs. Most people living with ALS use cough assist machines and feel more comfortable as a result. Regular use can help maintain the strength of your diaphragm and help prevent infections like pneumonia and bronchitis.

How does a cough assist work?

When your respiratory muscles are weakened, it can become difficult to cough strongly enough to draw phlegm from your lungs up to your mouth. The cough assist machine simulates a natural cough by applying positive pressure to inflate your lungs before using a vacuum-like suction to pull everything up.

Once secretions are in your mouth, you can use a suction machine to clear everything out.

How often should I use it?

You should use your cough assist regularly, even when you don’t feel you have anything in your lungs to clear. Regular use, such as once in the morning and once in the evening, serves as therapy to strengthen your diaphragm, keep your lungs clear, and reduce the chance of infection, which can be more difficult to recover from for people living with ALS. Each session produces multiple coughs and typically lasts between five and ten minutes.

When should I get one?

If you are experiencing a weak or impaired cough, your neurologist will order you a cough assist machine, often during a clinic or doctor visit. A durable medical equipment (DME) company will process the order through insurance and deliver the cough assist to you at home or at your ALS clinic. A respiratory therapist will train you and your caregivers how to use it and adjust your settings if needed.

How much will I have to pay?

Cough assist machines are considered rentals for the first 13 months and are covered through Medicare, Medicaid, and most private insurance plans. After 13 months, the machine becomes yours and your supplies will continue to be covered by your insurance. You may need to be proactive and ask your insurance for replacement masks, tubing, and filters ahead of time.

Suction Machine for ALS

Suction Machine

A suction machine is used to remove excess mucus and saliva that can accumulate in your mouth when your mouth and throat muscles become weaker and your swallowing may be impaired.

Clearing these secretions can help you breathe more easily, choke less when eating and drinking, and generally feel more comfortable. The suction machine is often used in conjunction with the cough assist machine, which pulls secretions from your lungs and throat up into your mouth for removal. The suction machine has an internal battery, so you can take it wherever needed.

How can I get one?

If you are having trouble managing secretions, your neurologist will order you a suction machine. No test is needed, and you do not need to meet any other specific criteria. A durable medical equipment (DME) company will process the order through insurance and deliver the suction machine to you at home or at your ALS clinic. A respiratory therapist will train you how to use and maintain it. Suction machines are often ordered in conjunction with bilevel machines.

How much will I have to pay?

Suction machines are fairly inexpensive. They are considered rentals for the first 13 months and are covered through Medicare, Medicaid, and most private insurance plans. After 13 months, the machine becomes yours and your supplies will continue to be covered by your insurance.

Invasive Mechanical Ventilation

When noninvasive ventilation no longer provides enough breathing support, invasive mechanical ventilation will be discussed as a way to extend your life. This requires tracheostomy surgery, which creates a circular hole in your windpipe at the base of your neck where the ventilator tube will connect. After that, the portable ventilator will breathe for you.

What would this mean for me?

Going on invasive mechanical ventilation means that you will need to be connected to your ventilator full-time and will need 24/7 care. You will receive nutrition through a feeding tube and will need to communicate through a speech-generating device.

What type of care would I need?

Once you are dependent on a ventilator, you will need 24/7 care. If you hire caregivers, they must be skilled, vent-trained professionals. This type of care is expensive, and is not covered by Medicare. Medicaid and some private insurance plans may pay for some level of care.

Family members can be trained to care for you. They will need to be fully trained before you return home from the hospital, which can take two to four weeks. Once at home, family caregivers will need to care for the trach site, maintain supplies, and suction secretions from the airway multiple times an hour.

How should I make this decision?

Getting a tracheotomy is a very personal decision. Talk with your neurologist, ALS clinic team, and a representative from your local ALS organization about the advantages and disadvantages of mechanical ventilation.

Talking about this with your loved ones is not easy, but it is extremely important. You may want to ask the social worker or nurse at your ALS clinic to facilitate the conversation, answer questions, and educate everyone on the realities of each option.

Though it is ultimately your decision, consider and discuss the impact it will have on everyone involved. Your loved ones may have strong feelings and differing opinions, and may even disagree with what you want. Try to explain your thinking and wishes as clearly as possible.

Once you make your decision, be sure to fill out advance directives to make your wishes very clear to your loved ones and medical professionals. This will provide the gift of clarity for everyone and help ensure that your wishes are followed.

Advance Directives for ALS

Advance directives are written documents that you can complete to let everyone know what type of medical treatment you would—or wouldn’t—want in the event of a medical emergency. If you become unable to make decisions, advance directives will make your wishes clear to your loved ones and to the medical professionals caring for you.

Why should I do this?

If you do not create advance directives ahead of time, your loved ones may find themselves in the challenging position of trying to make a decision for you. This can lead to confusion, guilt, and animosity. Advance directives can provide peace of mind for you and clarity for your loved ones.

It is very important to have a discussion with your loved ones and decide in advance whether or not you would want to have a tracheostomy procedure and be placed on invasive mechanical ventilation. If you do not discuss or write down your decision, the decision could be made for you in an emergency situation—and it may not be what you want.

Unwanted tracheotomies can and do happen. Medical professionals are obligated to do everything in their power to keep you alive—unless they have instructions from you stating otherwise.

Advance directives are simple forms. You do not need a lawyer to fill them out, though you can always seek legal advice if you’d like. You can change your advance directives whenever you’d like. The most important thing is to put your current decisions in writing so there is never any doubt about what you want.

Which forms should I fill out?

The three main types of advance directives for people living with ALS are a living will, a medical power of attorney, and a do not resuscitate (DNR) order. The official names of these documents can vary between states.

A living will is the core document that describes the type of life-prolonging support you would or wouldn’t want to receive, such as CPR, ventilation, a feeding tube, and pain management. A living will is different from the type of will that assigns property and money to loved ones upon death.

A medical power of attorney (POA), also called a health care proxy or durable power of attorney for health care, is a legal document that designates the person or persons you would like to make medical decisions on your behalf should you become unconscious or unable to make decisions. This person may be called a proxy, agent, surrogate, or representative.

Designating a medical power of attorney in addition to filling out a living will allows your proxy to communicate with medical professionals and make decisions in situations that may not be spelled out directly in your living will. If you have clearly communicated with your proxy, he or she should have a good idea of what you would or wouldn’t want.

It is important to choose a proxy you trust and who will follow your wishes, even if your loved ones strongly disagree. Ideally, this person can remain emotionally calm and stable in the event of an emergency. You do not have to choose a family member, and you may not designate a person who is on your medical team. You can choose more than one person, but if you do, you will need to designate which person will be in charge if both are present at the same time.

If you do not want first responders or medical professionals to attempt life-saving measures should you stop breathing or your heart stop beating, your doctor will need to sign a do not resuscitate (DNR) order for you. Your DNR will not be valid unless your doctor completes it and signs it.

Even if you already stated in your living will that you don’t want CPR or other life-saving measures, you should still fill out a DNR. In the event of an emergency, first responders and hospital staff will be obligated to try to save your life—unless they immediately see your DNR. They will not have time to look for and read your living will. Your DNR form should be posted in a visible place at home and ideally filed in your medical records.

Note: You may want to ask if you need a do not intubate (DNI) order in your state as well. Intubation is when a medical team puts a tube through your nose or throat if you are unable to breathe on your own. Intubation can lead to a tracheostomy, in which case you may wake up trached and vented, whether you wanted it or not. Clearly stating in your DNI or elsewhere that you don’t want intubation or mechanical ventilation will help avoid this scenario.

Where can I find these forms?

The social worker at your ALS clinic, local ALS organization, or local hospice should be able to give you the forms, help you fill them out, and answer your questions. Your hospital should have forms available, too.

You can also search online for advance directives in your state. The National Hospice and Palliative Care Organization makes it easy to find and download your state’s advance directive forms. They also provide state-specific information and step-by-step instructions that walk you through the process. The website e-forms has downloadable DNRs and downloadable living wills for each state.

Another option is called Five Wishes, which is a simple living will that gives you the freedom to express your most important wishes in a variety of areas, from medical and legal to personal and spiritual. It is a legal document that is accepted in most states. You can ask your local ALS organization or ALS clinic for a copy, or you can buy a copy from their website or complete it online.

If you spend a significant amount of time in another state, you may want to find out it if each state honors the other’s forms. If not, you may want to fill out advanced directives for both states.

You can also create your own advance directives form, but you will need to make sure you comply with your state’s requirements in order for it to be considered a legal document.

Every state has its own forms and laws about advance directives.

What else do I need to do?

We recommend starting with the social worker at your ALS clinic, local ALS organization, or local hospice. Review the forms together, ask questions, and discuss your options. You may also want to meet with your neurologist and ask what could happen in various medical scenarios.

Though this can be a difficult topic to discuss with your loved ones, it is important to explain your decisions and make sure that everyone understands your wishes, even if not everyone agrees. If you are worried about initiating this conversation, ask your social worker to facilitate, answer questions, and educate everyone.

Once you have made your decisions, you will need to fill out the forms and sign them with a witness present, and possibly a notary, depending on your state’s laws. If you comply with your state’s requirements, your forms will become legal documents when you sign them.

Give copies to your proxy, loved ones, medical teams, and the hospital where you would go in the case of an emergency. Keep your original in a safe place that others know about and can access in the case of an emergency. Other options for making sure your wishes are shared include emailing copies, storing them on the cloud, keeping a card in your wallet, traveling with your documents, and having a flash drive with you.

Also be sure to tell your loved ones who you have designated as your proxy. Explain that this person’s role is to carry out your wishes.

Traveling with Respiratory Devices

Traveling with respiratory equipment is possible, though it does require advance planning. Make sure you leave home with everything you need, such as extra batteries, power converters, adapters, extension cords, masks, and tubing.

Can I travel by plane with my equipment?

Yes. Most respiratory devices are FAA-approved. Check your device manual or consult with your respiratory therapist to make sure. Contact your airline—ideally a few weeks in advance—to ask if you need approval to bring your device(s) on the plane. Bring all related paperwork, such as your neurologist’s prescription for each device.

Your respiratory devices should not be considered carry-ons and can be stored in the overhead bin. You can use your respiratory devices as needed while on the plane. Make sure you have enough battery-backup time for unexpected delays. You can ask ahead of time if there will be power outlets on the plane.

Talk with your respiratory therapist ahead of time about your travel plans. Ask for tips, ideas, and concerns regarding traveling with your specific devices.

Keep in mind that you will need to be able to sit in a standard airplane seat for the duration of the flight.

How portable are all the devices?

Bilevel machines, used primarily for sleeping, must be plugged into an outlet—unless you purchase an external battery out of pocket. Portable ventilators, multifunction ventilators, cough assists, and suction machines all have internal batteries, which means you can use them while you’re on the move, whether in a car, bus, train, or plane. If you have a power wheelchair, your portable ventilator can plug into your wheelchair battery, if needed.

Ventilator & Tracheostomy Decision

One of the decisions that a person living with ALS will face on their journey are respiratory options. As ALS progresses and breathing becomes more challenging, it is a good idea to have a conversation about respiratory choices and wishes. When facing respiratory decisions in ALS, there are many factors to consider.

Non-invasive ventilator support- this refers to a machine that assist you with breathing, such as a BI-Pap or Trilogy. This is referred to as NIV and is typically a mask or nose pieces that is non-invasive.
Tracheostomy & ventilator support- this is a surgical procedure which consists of opening a direct airway through an incision in the trachea and often referred to as “invasive” ventilation. This machine does the breathing for you.

Here are some important things to remember as you navigate these decisions:

Invasive ventilation does not stop disease progression. Even if a person opts for a tracheostomy, the ALS will continue and muscles will continue to weaken. A patient using invasive ventilation may live another 5-15 years.
The amount of care required for a person on a vent is quite intense. A person living with ALS who has had a tracheostomy and uses a ventilator cannot be alone and requires 24/7, round-the-clock care.
Hospice is not an option for patients who have chosen a tracheostomy & ventilator; and it is very difficult to find home health companies that are able to assist with vent care. The continuous care usually falls on the family, and the out of pocket cost for private care nurses generally runs from $75+ per hour.
There are only a limited number of facilities that can accept people who are on invasive ventilation. Living on a vent long term in a care facility can cost more than $10k per month, and this is a cost that is not covered by Medicare.

Considerations when deciding on invasive respiratory support:

The decision to move to invasive ventilation, or living on a trach, is a very individual part of the journey with ALS, both for the patient and the caregiver. The individual living with ALS must face several choices, such as what does quality of life mean for them and end of life decisions. The person providing care also has a choice on whether or not they are able to commit to that level of care.

Will your medical insurance cover the cost of a mechanical ventilator, portable suction unit, secretion mobilization devices, and all disposable supplies in the home?
Who will provide 24-hour care for you in the home?
If family members, will they be comfortable managing a mechanical ventilator and your tracheostomy care and suctioning secretions from your lungs? Secretions are a common part of living on a trach, and the degree of secretions experienced is different for each person. For the first six weeks or so after the trach/vent are placed, the suctioning is worse and seems to taper off after the initial six-week time frame. For some people, this may look like suctioning 4-6 times a day, but for others it may be suctioning every 15 minutes or more.
Will family care providers have the physical and emotional support systems to help them help you?
Will your medical insurance pay for in-home help to assist with ventilator, tracheostomy, suctioning, bathing, feeding, dressing, and other necessities of daily life?
If insurance does not cover this cost and you do not have enough family members to provide 24-hour care, do you have the financial resources to pay for nursing care?
Is there a medical home care equipment company near your home that will provide the mechanical ventilators and respiratory therapists to train family and home?
How is the ability to communicate maintained with others as my speech declines? It is vital to have a plan for communication in place when on mechanical ventilation.

Withdrawal of care, or ventilator support, is an important decision to keep in mind when considering getting a tracheostomy and a ventilator. A person living on invasive ventilator support may need to make the decision as to when to stop using the vent, which can be a difficult process to go through mentally and emotionally. There are many emotions to process as a person makes the decision as to where their line regarding quality of life is, and when to stop care. This can often be a painful process for the family as well. When the choice is made to stop mechanical respiratory support, Hospice is available to help the person be comfortable and support the family.

Tracheostomy Patient Education

ALS can affect your breathing muscles, including the diaphragm and chest wall muscles. These muscles work together to take air into the lungs and push it back out. With ALS, there is nothing wrong with your lungs and absorbing oxygen from the air! The issue is muscle weakness and just getting the air in and out. As ALS progresses, you and your physician may discuss a tracheostomy and mechanical ventilation. The decision to proceed with a tracheostomy and mechanical ventilation is a very personal decision. There is not a right or wrong decision. Discuss the logistics of this decision with your care team, family members and caregivers. Ultimately, the decision is up to the person living with ALS.

What is a tracheostomy?

A tracheostomy (also called a “trach”) is a surgical procedure to create an opening in the front of the neck into the trachea (windpipe). A tracheostomy tube is placed through the hole and into the windpipe. A mechanical ventilator (breathing machine) is then attached to the tracheostomy tube and does the work of breathing. This is called invasive ventilation.

Why would I need invasive ventilation?

There are multiple reasons that invasive ventilation is chosen. Most commonly it is because non-invasive ventilation (AVAPS or Trilogy) is no longer providing adequate respiratory support. Additionally, if there is a lot of difficulty with secretion management making non-invasive ventilation with a mask unsafe, then invasive ventilation may be considered.

What are the advantages of having a trach?

• Longer survival
• Rests respiratory muscles (less work to breath which helps with energy conservation)
• Easier to clear lung secretions
• More secure ventilation than non-invasive ventilation
• No Mask

What are the disadvantages of having a trach?

• Increased need for suctioning
• Increased chance for mucous plugging in the lungs
• Increased risk for respiratory infections
• Safety concerns (potential malfunction of the ventilator or unexpected disconnection)
• Need for 24/7 caregiving
• Financial resources can often be depleted by the cost of care
• Not all residential care facilities are willing to take ventilator patients

Will getting a trach slow down my ALS progression?

No, ALS will continue to progress at the same rate. A trach and mechanical ventilation only provides respiratory support. A trach can prolong your life but does not have any effect on other parts of your body beyond the lungs.

Characteristics of those who do best after getting a trach:

• Highly motivated and engaged in life
• Full understanding of the advantages/disadvantages
• Able to communicate (can be through the use of a communication device)
• Active family and support system in place that is willing to help
• Financial resources for equipment and caregiving

What happens after the trach surgery?

Expect to be in the hospital for multiple weeks. Often, patients will be transferred to rehabilitation hospital (such as Vibra) after 1-2 weeks in the hospital to continue family education and healing. Family and caregivers will be trained prior to discharge home by both the hospital staff and respiratory company. The respiratory company will have to complete a home safety/electrical evaluation prior to discharge as well.

I heard that 24/7 caregiving is necessary, who is an appropriate caregiver?

It is critical to have 24/7 caregiving for a person on a trach/mechanical ventilation. They are needed for suctioning and for any emergencies. A caregiver can be any responsible persons that feel up to the task of caring for a person with a trach. Your respiratory company can train any responsible person on providing care and understanding the ventilator alarms. Caregiving agencies are an option for additional caregiving support but can be expensive and is not always covered by insurance. It is possible to move into a foster home but there are very few beds available so placement can be difficult. Some skilled nursing facilities with take patients on ventilators.

Does a trach hurt?

There can be discomfort/pain immediately following the surgery. However, once the trach site heals, there is normally not any discomfort. If there is discomfort around the stoma, it could be irritation or an infection which can both be easily treated.

Do you have to change the trach tube and why?

The trach tube is typically changed every 3 months. If there is a lot of granulation tissue, it may need to be done more frequently. The first change is completed in the hospital settings to ensure there are no complications. After the first change, the trach tube can often be changed in the home and is a very swift process that caregivers can be trained to complete. Routine trach tube changes can prevent infection and prevent granulation tissue formation.

What does a normal day like?

Daily care:
• Cleaning around the trach at least once daily (more if there is redness/drainage)
• Cleaning inner cannula at least daily (more if there is a lot of secretions)
• Change trach ties regularly
• Oral suctioning for saliva
• Trach suctioning to remove secretions/mucous that can’t be coughed up (this is frequent throughout the day)

What equipment is necessary with a trach?

Your respiratory company will instruct you on cleaning and ordering supplies. Follow a regular cleaning schedule for all equipment to ensure it remains functional and safe. Always stay on top of supply ordering so you don’t run out!

• Ventilator (Trilogy can be used with a trach and different attachment)
• Humidifier (works in line with the Trilogy
• Suction machine (used for both trach and oral suction)
• May need : Cough Assist or Vest (high frequency chest wall oscillation)

Emergencies sound scary, what can I do to be safe?

Emergencies are not common but there are things to be prepared for. Check with your respiratory company to see if a back-up ventilator and/or batteries are an option. These come in handy if the ventilator malfunctions or the power goes out. It is also a good idea to have a back-up generator in case of a power outage. Have your respiratory company’s 24 hour on-call number readily available (put it right on the ventilator!). Have extra supplies on hand to fix problems in the home.

What happens if the trach tube comes out?

Always keep an extra trach within reach for this emergency. You will be trained on how to respond in this type of situation and how to put the extra trach in. Typically, the spare trach is a size smaller to ensure that it can be placed easily in an emergency.

Can I talk with a trach?

Most persons with ALS and a trach cannot speak. However, if a person could speak just prior to getting the trach, they may be a candidate for a speaking valve that will allow for speech. However, the disease will still progress and bulbar function will likely decrease with time. It takes strength to use the speaking valve which can use up valuable energy.

Can I eat with a trach?

Most persons with ALS and a trach do not eat by mouth. However, (similar to talking) if the person is in early stages of ALS and still has good throat and mouth muscle tone, they may be able to eat with a trach. The cuff of the trach can make it difficult to swallow. If there is swallowing issues, it is not safe to eat with a trach. If the food is aspirated, it will enter the windpipe and stay on the cuff. Once the cuff is deflated, any aspirated contents will enter the lungs. Typically, a feeding tube is placed during the same hospital stay as a trach placement if it was not already in place.

What happens if I cough? Does the trach come out?

The trach tube is securely held to the neck with the use a trach tie so it will not come out as long as the trach tie remains secure. If you are able to cough, the secretions can be suctioned out of the trach tube. If you have difficulty coughing, a cough assist can be used.

Can’t I just use oxygen to help me breathe better?

ALS affects the respiratory muscles that control breathing function. As the respiratory muscles weaken, it is difficult to move air in and out of the lungs. The problem is not an oxygenation problem but a ventilation problem. Providing supplemental oxygen without ventilator support can cause or accelerate respiratory distress. Oxygen should only be used by doctor recommendation. You made need supplemental oxygen if you have a pre-existing condition outside of your ALS or if your oxygen saturation levels fall below 90%.

How do I shower with a trach?

It is possible to take a shower with a trach as long as EXTREME care is used. Water/soap must not get into the trach opening. There are trach bibs that cover the opening. You can also have sponge baths and use a shampoo wash basin.

Can I still have pets if I have a trach?

Yes, you can have pets as long as you take extra precautions to stay safe. Keep shedding minimal by adopting hypoallergenic pets, brushing pets daily and vacuuming floors often. Keep supplies and tubing out of reach of animals that might chew on them or curious kittens. Keep the pet away from the trach site.

Will I still be able to leave my home?

Have a trach and ventilator does not mean that you have to be stuck at home! The ventilator can be attached to the back of a power wheelchair. It is important to think about how you will travel once you have a ventilator and consider a power wheelchair van or setting up medical transport. Bring extra supplies with you and back-up batteries. If you are travelling by plane, ask your respiratory company for your ventilator FAA approval to show security. Keep a copy of your ventilator settings and the number of respiratory company with you when leaving the home. Remember that your ventilator is on the back of your power wheelchair when turning or going through doorways.

How can I stay healthy?

Oral care is very important for overall health. Good oral care can prevent pneumonia and suctioning can prevent excess secretions from entering the windpipe. Be sure to provide oral care at least twice a day and visit the dentist regularly. Always get your flu shot and pneumonia shot (caregivers too!)! Ensure that all caregivers and visitors wash their hands before touching you. Ask your family and friends to stay away if they do not feel well.

Can I have sex while on a ventilator?

With ALS, the desire and function to have sexual relations is usually intact. Ventilator tubing and decreased mobility of the person with ALS can make sex more challenging. However, with creativity and careful consideration, it is still possible. Intimacy can also be expressed through touching and caressing as well if intercourse proves challenging.

How do people with a ventilator die?

Some people on ventilators pass away from respiratory infections that become severe. There are rare occasions when a person on a ventilator dies from the ventilator malfunctioning or disconnecting and there is not someone present to help. It is important to have the difficult discussion of when you would want to have ventilator support removed. There are multiple points at which the person with ALS can decide to remove their ventilator. Once communication is very difficult, with recurring pneumonias that are difficult to treat or with the onset of dementia are all times that some people decide they would want the ventilator support removed. It is important to discuss your wishes with your healthcare team and your family and friends so your wishes can be supported. If you are mentally capable, you can decide at any time to remove ventilator support. Not discussing your wishes with your support system can result in a quality of life that you do not want.

Is it uncomfortable to remove ventilator support?

If you decide that you want to remove your ventilator, we will arrange for hospice to be present. Hospice can provide medications to make the process very comfortable and peaceful. Withdrawing ventilator support will lead to death typically within hours of the disconnection.

Providence ALS Center 5.3.19

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