Spinal muscular atrophy (SMA) is an inherited disorder that causes 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 doctor specializing in respiratory management) can help develop a respiratory care plan depending on the type of SMA and severity of breathing problems a person has. The plan may involve several action items and treatments for SMA, including 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.

Who Uses BiPAP?

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.

Many people with SMA types 2 or 3 can manage 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.

How Does BiPAP Work?

In people with SMA, the intercostal muscles found between the ribs are very weak. These muscles help facilitate breathing by allowing the chest to expand and fill the lungs with air. Because SMA weakens the intercostal muscles, it can compromise a person’s ability to maintain a normal breathing pattern.

Breathing problems can lead to:

• Weakened and underdeveloped lungs
• Weak cough
• Severe risk from viral respiratory infections, even those that would cause only minor illness in people without SMA
• Increased risk of pneumonia
• Shallow breathing (hypoventilation), especially during sleep
• Aspiration (inhalation in the lungs) of food and drink

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 are getting enough air when you are in a deep sleep and are not breathing well enough on your own.

Is Using BiPAP for Spinal Muscular Atrophy Safe?

According to Johns Hopkins Medicine, BiPAP is usually very safe to use. Whereas an invasive breathing-support procedure, like a tracheostomy, might expose the individual to infection, BiPAP has a lower risk of complications.

The main problems that people may experience while using a BiPAP device involve the face mask. It may fit too tightly, 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.

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.

Other potential risks of BiPAP include:

• Dry mouth
• Eye irritation
• Leaking from the mask, which may decrease the air pressure being delivered
• Sinus pain or congestion
• Mild stomach bloating

Some of these side effects are treatable, fortunately. 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 your doctor about reducing the pressure setting of the machine.

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.

Getting Started With BiPAP for Spinal Muscular Atrophy

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 BiPAP machine.

Following are some factors to keep in mind as you get started with BiPAP.

Find the Right Equipment

Try on several masks before deciding on one. You might still need to make adjustments to the one you like to ensure it fits properly.

Get To Know Your BiPAP Machine

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.

Calibration

Before you use your BiPAP machine, an expert such as a respiratory therapist may need to calibrate it; that is, adjust the settings to fit your needs. The expert may also give you additional instructions on how to use the machine, including how to prepare yourself and how to maintain the device.

Adjust To Using BiPAP

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).

Let your doctor 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.

Find Your Support System

On mySMAteam, the social network for people with spinal muscular atrophy and their loved ones, more than 2,600 members come together to ask questions, give advice, and share their stories with others who understand life with SMA.

Have you used BiPAP or another type of ventilation with SMA? Share your experiences in the comments below, or start a conversation by posting on mySMAteam.