Motor neurone disease (MND) causes the muscles you have control over to weaken- this includes the muscles involved in breathing.
Two main types of voluntary muscles are involved in normal breathing:
Both sets of muscles are called respiratory muscles. When you contract respiratory muscles, they lift and expand your rib cage, pulling air into your lungs. Relaxing the muscles expels air from your lungs. This process provides the opportunity for your lungs to take the oxygen from the air that your body needs for survival and breathe out an unwanted gas called carbon dioxide that is produced by the body.
The blood carries oxygen from your lungs to cells throughout the body, where it plays an important role in how your body works.
Seeking regular assessment and advice about your breathing as soon as possible after your diagnosis, even if you have not noticed any changes in your breathing, is important.1
This will help you get the advice and information you need, discuss options with your healthcare team and decide which strategies are right for you.
Increasingly, many people with MND are choosing to use non‐invasive breathing support, also known as non‐invasive ventilation (NIV) or bi-PAP when breathing muscles become weak. NIV involves wearing a mask over the nose, and/or mouth and nose, connected to a small pump (bi-pap machine) that creates just the right amount of pressure to keep your airways open so that room air can easily come in and out of your lungs when you breathe.
The NIV machine is usually used at night but as the respiratory muscles weaken further you might use it at times during the day as well.
A cough is a very important function and can be thought of as a powerful breath out that helps keep your airways clear.
When your cough is weak you may be more likely to get chest infections or have difficulty in getting rid of excess saliva.
A number of factors can cause problems managing saliva. These can include changes to swallow function, medications, diet, breathing or positioning. These changes can lead to a build-up of thin or thick saliva and can lead to significant distress and discomfort.
A speech pathologist, physiotherapist or doctor can provide you with advice and more information about saliva management and also regarding techniques to improve cough effectiveness.
Some techniques that may help you cough more effectively include:
A weak cough can make it more difficult for you to clear thin liquids or thick and chunky foods that may accidently get caught in your throat during eating and drinking. Your speech pathologist and dietitian can provide you with advice and recipes for foods and drinks, including advice about thickening agents. Your physiotherapist can show you and your carer how to use an 'assisted cough' to clear your airways in case food goes down the wrong way.
If you are undernourished your muscles, including your respiratory muscles, will be weaker. This may increase your susceptibility to coughs and colds. Maintaining a healthy diet with the right mix of proteins, carbohydrates and other nutrients, is important.
A well-balanced diet, with enough fluids, will also help you avoid getting constipated. If you become constipated, more effort is required to empty your bowel. Because your diaphragm is a muscle which also helps you to push down and empty your bowels, respiratory muscle weakness can affect how well you empty your bowels.
Your dietitian, community nurse or general practitioner can advise you about specific dietary issues and constipation.
The way you position your body while sitting or lying down can assist your breathing. Electric recliner chairs, adjustable beds and wheelchairs enable you to easily experiment with a number of different positions. There are different positions and support pillows and aides that can assist you in finding your preferred position to make breathing more comfortable. Your physiotherapist or occupational therapist can advise you about positioning and where to get equipment.
Breathing exercises may help your lungs to expand more fully, and in turn reduce pooled air in the lungs. One simple exercise is to take five to ten deep breaths, with short rests in between each breath, several times a day. Speak with your physiotherapist, palliative care team, specialist respiratory nurse or respiratory physician about the right breathing exercises for you.
Having an open window in the room and using a fan to circulate air can also assist your breathing.4 Room temperature is important as being too hot or too cold can make you feel uncomfortable. A humidifier may help increase the moisture in the room air and increase your comfort.
Avoid people with coughs and colds. Have an influenza vaccine before winter to reduce the risk of getting the flu. Your doctor or respiratory physician may also recommend a pneumonia, or other vaccines.
Fatigue can be a major problem for people with MND. There is no advantage in ‘pushing yourself’. Instead, try and save your energy for activities that are most important for you.
Things that may help include:
Respiratory nurses, physiotherapists, occupational therapists, rehabilitation specialist staff and other health professionals can teach you how to save your energy, and offer advice on equipment and labour-saving devices.
Anxiety or worry about breathlessness can affect how well you breathe. Controlled breathing and other techniques can help you to relax and make breathing easier. A calm, confident approach by your carer is also helpful. Your physiotherapist, palliative care team, specialist respiratory nurse and respiratory physician can advise you about the right techniques for you.
Other strategies for managing breathing with motor neurone disease include medically supervised use of medications. Your GP or neurologist can provide further input with regards to what medication will work best in your situation.