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Spinal muscular atrophy (SMA) is a health problem caused by genes passed down from parents. It leads to muscle weakness and wasting over time. Some forms of SMA can cause the respiratory muscles (muscles involved in breathing) to be weaker than usual, leading to breathing problems. Breathing problems are the leading cause of illness for people living with SMA, making breathing support important.
A pulmonologist (a lung expert) can help develop a respiratory care plan depending on a person’s type of SMA and how bad the breathing problems are. The plan may involve several action items and SMA treatments, including using breathing support, also called ventilation or mechanical ventilation.
A common type of respiratory equipment for people with SMA is bilevel positive airway pressure (BiPAP). BiPAP machines help open and push air into the lungs through what’s known as the positive pressure ventilation method.
BiPAP machines come with a range of features, which are described below in more detail. Your healthcare provider will conduct an assessment and help you figure out what type of machine might be best for you or your loved one’s condition.
A pulmonologist may recommend ventilation using a BiPAP machine for certain people who have breathing problems as a result of SMA.
Ventilators are machines that help you breathe or breathe for you. These machines can either be invasive or noninvasive. BiPAP is a type of noninvasive ventilation. It aids breathing using an airtight mask placed over the nose or the nose and mouth, or through a tube under the nose and into the nostrils.
Your SMA type plays a big part in determining the best mechanical ventilation for your situation. Many people with SMA types 2 or 3 can manage well with noninvasive ventilation, such as a BiPAP machine. They may only need this type of ventilatory support when sleeping or ill. For example, the common cold can weaken breathing in children with SMA. According to Cure SMA, if a child with SMA who already uses BiPAP comes down with a cold, they will need to use the machine during all sleep, including daytime naps, and occasionally while awake.
Some children and adults with SMA type 3 may need frequent checkups to look for types of breathing trouble that are more difficult to recognize, such as obstructive sleep apnea and hypoventilation (shallow breathing). Using BiPAP during sleep may help these people.
Children with SMA type 1 may also benefit from BiPAP breathing support during sleep, but they may also need 24-hour support or invasive ventilation. A review of several studies showed that BiPAP improved sleep quality and increased survival rates for children with SMA type 1.
In people with SMA, the intercostal muscles found between the ribs are very weak. These muscles help with breathing by allowing the chest to expand and fill the lungs with air. Because SMA weakens these muscles, it can affect lung function and compromise a person’s ability to maintain a normal breathing pattern.
Untreated breathing problems can lead to:
A BiPAP machine helps you breathe by using positive pressure to push air into the lungs. The machine provides two different levels of positive airway pressure: When you breathe in, the device provides higher pressure, allowing you to take in an increased volume of air. During exhalation, the pressure lowers automatically. BiPAP machines can sense your normal breathing cycle and synchronize with it — that is, work at the same time or rate.
A BiPAP machine can also help ensure you’re getting enough air when you are in a deep sleep and are not breathing well enough on your own. It helps make sure your lungs are getting enough oxygen to function correctly.
According to Johns Hopkins Medicine, BiPAP is usually very safe to use. In addition to the noninvasive nature of BiPAP treatment, it comes with a lower risk of complications compared to an invasive breathing-support procedure, like a tracheostomy.
Your doctor will help you determine if a BiPAP machine is the best strategy to improve your breathing. It’s possible BiPAP might not be right for you if you have very poor breathing, trouble swallowing, or if you sometimes lose consciousness.
Importantly, you should never eat or drink while using your BiPAP machine. Doing so could cause you to inhale food or beverage into your lungs.
Your individual risk may vary depending on your age, the amount of time you use the BiPAP device, and any other medical conditions. Talk to your doctor if you experience any problems or have any concerns about using BiPAP.
There are several different features associated with BiPAP devices. Your healthcare provider can help you decide what type of machine is best for you. Your pulmonologist or respiratory therapist may conduct a breathing function test or a sleep study to help determine which device and settings help you breathe most easily. During a sleep study, a technician monitors your breathing and measures things like oxygen, airflow, and carbon dioxide, and adjusts the BiPAP settings to determine the best settings to maximize your respiratory function.
Your BiPAP machine provides airway pressure in one of three ways:
Consult with your healthcare team to see which method might be best for you. Try on several masks before deciding which one to use. You might still need to make adjustments to the one you like to ensure it fits properly.
BiPAP devices have two different pressure settings — one for when you’re inhaling, or taking in air, and another lower pressure setting for when you’re exhaling, or blowing out air. The pressure settings may be either preset to specific intervals based on your natural breathing rhythm (timed) or they may adjust automatically to your real-time breathing patterns (automatic).
Your respiratory specialist will help you figure out which setting is best for your lung function.
Some models have a heated humidifier, which might be helpful if you experience dry mouth.
Your pulmonary specialist will give you instructions on how and when to use your BiPAP machine. Following their instructions will allow you to get the most benefits from your medical equipment.
Here are some things to keep in mind as you get started with your BiPAP device:
It’s a good idea to familiarize yourself with the different parts and features of your BiPAP machine, including its mask or nasal plugs, the motor that delivers air, and the tubing connecting the motor to the mask or plugs. Your machine may also have additional features, such as a heated humidifier.
Before you use your BiPAP machine, an expert such as a respiratory therapist may need to calibrate it, meaning adjust the settings to fit your needs. The specialist may also give you additional instructions on how to use the machine, including how to prepare yourself for BiPAP therapy and how to maintain the device.
Getting accustomed to using a BiPAP machine can take time as you adjust to wearing the mask or nasal plugs, feeling the flow of air, and getting used to its sounds (which should be soft). Making sure the mask fits correctly is important, as an ill-fitting mask can contribute to poor results.
Let your care team know if you feel that you can’t breathe while using your BiPAP device. They may need to adjust your machine’s pressure settings. If the noise from the machine bothers you, using earplugs might help. If the noise is too loud, check with your supplier to ensure your BiPAP machine is working properly.
The main problems that people may experience while using a BiPAP device involve the face mask: It may feel too tight, causing discomfort or skin damage where it sits on the face. Check to make sure the straps around your mask are adjusted properly. You may also need a different size or type of mask.
Other potential side effects of BiPAP include:
Some of these side effects are treatable. Consider using a humidifier to reduce dryness in your nose. For sinus congestion and headaches, your doctor may prescribe antihistamines. If you experience stomach bloating, talk to a healthcare professional about reducing the pressure setting of the machine.
On mySMAteam, people share their experiences with spinal muscular atrophy, get advice, and find support from others who understand.
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I have Kennedy's Disease (X linked, spinal bulbar muscular atrophy) and have been using a BiPAP for the past 8 months. It took about a month to get use to it but after that my sleep has greatly… read more
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