When someone has ALS, their muscles slowly get weaker over time. This includes the muscles that help us breathe. The breathing muscles are some of the most important muscles in our body, so taking care of them is a big part of managing ALS.
Learning about respiratory care might seem scary at first, but understanding how to support your breathing can help you feel more in control. This guide will walk you through the basics of ALS respiratory care, from simple breathing exercises to more advanced equipment that can help when breathing becomes harder.
Understanding How ALS Affects Breathing
To understand respiratory care, it helps to know how ALS changes the way we breathe. Normally, when we breathe in, our diaphragm moves down and our chest muscles expand to pull air into our lungs. When we breathe out, these muscles relax and push the air back out.
In ALS, the nerve cells that control these muscles stop working properly. This means the muscles that help us breathe can become weak. The diaphragm, which is the main breathing muscle under our lungs, might not move as well as it used to. The muscles between our ribs, called intercostal muscles, might also get weaker.
When these muscles don't work as well, several things can happen. You might feel short of breath, especially when you're active or lying down. You might notice that you can't take as deep a breath as before. You might also have trouble coughing strongly enough to clear mucus from your lungs.
These changes don't happen all at once. For many people with ALS, breathing problems develop slowly over months or years. This gives you and your healthcare team time to plan and prepare for respiratory care.
Early Signs to Watch For
Knowing the early signs of breathing changes can help you get the right care at the right time. Some of the first signs might be subtle and easy to miss.
You might notice that you feel more tired than usual, especially in the morning. This could be because your breathing muscles are working harder at night when you're lying down. Some people find that they wake up with headaches or feel like they didn't sleep well, even if they were in bed for eight hours.
Shortness of breath during activities that used to be easy is another early sign. This might happen when you're walking up stairs, carrying groceries, or even just talking for a long time. You might find yourself needing to take breaks more often or feeling winded after activities that never bothered you before.
Changes in your voice can also be an early sign. Your voice might sound weaker or more breathy than usual. You might find it harder to project your voice or speak loudly enough for others to hear you clearly.
Some people notice that they have trouble lying flat in bed. You might find yourself needing extra pillows to prop yourself up, or you might prefer sleeping in a recliner chair instead of a regular bed.
The Importance of Regular Monitoring
Once you know you have ALS, your healthcare team will want to check your breathing regularly. This usually involves simple tests that measure how well your lungs are working.
One common test is called spirometry. During this test, you breathe into a machine that measures how much air you can breathe in and out, and how fast you can do it. The most important number from this test is called FVC, which stands for forced vital capacity. This tells your doctor how much air you can push out of your lungs after taking the deepest breath possible.
Another important measurement is called maximum inspiratory pressure (MIP). This test measures how strong your breathing muscles are when you try to suck air in. Your healthcare team might also check your maximum expiratory pressure (MEP), which measures how strong your muscles are when you push air out.
These tests help your healthcare team understand how your breathing is changing over time. They use these numbers to decide when you might benefit from different types of respiratory support.
Your doctor will probably want to do these tests every few months. Don't worry if the numbers go down over time — this is expected with ALS. The important thing is that your healthcare team knows what's happening so they can help you get the right support when you need it.
Non-Invasive Ventilation: Your First Line of Support
When your breathing muscles start to get weaker, the first type of support your doctor might recommend is called non-invasive ventilation (NIV). The word "non-invasive" means that it doesn't require surgery or putting anything inside your body.
The most common type of NIV is called BiPAP, which stands for bilevel positive airway pressure. A BiPAP machine is about the size of a small shoebox and sits next to your bed. It connects to a mask that fits over your nose, or sometimes over your nose and mouth.
The BiPAP machine works by gently pushing air into your lungs when you breathe in, and then allowing you to breathe out normally. This helps your tired breathing muscles by doing some of the work for them. Many people find that using BiPAP helps them sleep better and feel more rested during the day.
At first, you might only need to use BiPAP at night while you're sleeping. This is because lying down makes it harder for weak breathing muscles to work properly. As time goes on, you might find it helpful to use BiPAP during the day too, especially when you're resting or feeling short of breath.
Getting used to BiPAP can take some time. The mask might feel strange at first, and the feeling of air being pushed into your lungs might seem odd. Most people find that it gets more comfortable after a few nights of practice. Your respiratory therapist can help you find the right mask and adjust the settings to make it as comfortable as possible.
Cough Assist: Keeping Your Lungs Clear
One of the most important jobs your breathing muscles do is help you cough. Coughing is your body's way of clearing mucus and other things out of your lungs. When your breathing muscles get weak, you might not be able to cough strongly enough to keep your lungs clear.
This is where a cough assist device can help. A cough assist machine, sometimes called a mechanical insufflator-exsufflator, helps you cough more effectively. It works by first pushing air into your lungs, and then quickly sucking it back out. This creates the same effect as a strong, natural cough.
Using a cough assist device is usually not uncomfortable. It connects to a mask that fits over your nose and mouth, similar to a BiPAP mask. The machine does a series of breaths for you, and then helps you cough out any mucus that might be in your lungs.
Your healthcare team will teach you how to use the cough assist device and help you figure out when to use it. Some people use it every day as part of their routine lung care. Others might only need it when they have a cold or feel like they have extra mucus in their lungs.
Keeping your lungs clear is very important for staying healthy with ALS. Mucus that stays in your lungs can lead to infections, which can be serious for people with breathing muscle weakness.
Building Your Respiratory Care Team
Taking care of your breathing with ALS is a team effort. Your respiratory care team might include several different healthcare professionals who each have special training in helping people breathe better.
Your pulmonologist is a doctor who specializes in lung and breathing problems. They will monitor your breathing tests and help decide when you need different types of respiratory support. They work closely with your ALS doctor to make sure all of your care fits together well.
A respiratory therapist is someone who specializes in helping people use breathing equipment. They will teach you how to use devices like BiPAP and cough assist machines. They can also help you troubleshoot problems and make adjustments to make the equipment more comfortable.
Your ALS clinic team will also be involved in your respiratory care. This might include nurses, social workers, and other specialists who understand how ALS affects breathing. They can help coordinate your care and make sure you have everything you need.
Don't forget that your family and caregivers are also important members of your respiratory care team. They can learn how to help you use your equipment and watch for signs that you might need extra support.
Planning Ahead: Making Decisions About Advanced Care
As ALS progresses, you might need to make decisions about more advanced types of respiratory support. Having these conversations early, when you're feeling well, can help you make choices that match your values and goals.
One option that some people choose is a tracheostomy with mechanical ventilation. This involves a small surgery to create an opening in your neck where a breathing tube can be placed. The breathing tube connects to a ventilator machine that can do all of the work of breathing for you.
This type of support can help people with ALS live longer, but it also requires a lot of care and support from family members or professional caregivers. It's a very personal decision that depends on many factors, including your goals, your family situation, and your access to care and support.
The important thing is to have these conversations with your healthcare team and your family before you need to make urgent decisions. Your ALS team can help you understand all of your options and support you in making choices that are right for you.
Staying Active and Maintaining Quality of Life
Having respiratory support doesn't mean you have to stop doing the things you enjoy. Many people with ALS continue to travel, spend time with family and friends, and pursue hobbies even while using breathing equipment.
Modern respiratory equipment is designed to be portable. Many BiPAP machines can run on battery power, and some are small enough to take with you when you travel. Cough assist devices are also becoming more portable and easier to use.
Your respiratory therapist can help you learn how to use your equipment in different situations. They might teach you how to use a manual resuscitation bag as a backup, or show you how to clean and maintain your equipment while you're away from home.
Staying active within your abilities is important for both your physical and mental health. Gentle exercises, even just moving your arms or doing breathing exercises, can help keep your remaining muscles strong and your lungs as healthy as possible.
The Bottom Line
Respiratory care is a crucial part of managing ALS, but it doesn't have to be overwhelming. By working with your healthcare team, learning about your options, and planning ahead, you can maintain your quality of life and stay as healthy as possible.
Remember that respiratory support is meant to help you feel better and do more of the things you want to do. Don't hesitate to ask questions, try different equipment options, or ask for help when you need it. Your respiratory care team is there to support you every step of the way.
The most important thing is to stay connected with your healthcare team and let them know how you're feeling. With the right support and equipment, many people with ALS are able to manage their breathing symptoms and continue living full, meaningful lives.
