Spinal muscular atrophy: Breathing support options

A variety of methods and devices can make breathing and coughing easier.

Breathing (respiratory) problems are a significant issue for people with the most common types of spinal muscular atrophy (SMA), especially types I and II. SMA damages nerve cells in the spinal cord and causes muscle weakness — including muscles in the neck, throat, chest and rib areas, which are essential for breathing.

These muscles are also used for coughing, a necessary action to clear mucus from the lungs and airways. Mucus increases during respiratory illnesses like the common cold and respiratory syncytial virus (RSV). When a person is unable to cough, the mucus can make breathing more difficult. Mucus can also settle into the lungs and lead to pneumonia infections.

Breathing support (ventilation) options include methods that don't require any equipment, tubes or needles, to enter the body (noninvasive). Children with less severe symptoms, like those with SMA type III, may only need this type of breathing support.

But children with more-severe symptoms, like those who have SMA types I or II, may also need breathing support that uses equipment that enters the body (invasive). Breathing problems cause most of the illnesses and deaths in children with SMA types I and II. But breathing support can significantly help those children to live longer and better.

It's important to understand breathing support options and talk to your care team regularly about your needs.

Noninvasive breathing and coughing support

Noninvasive devices and methods can help strengthen lungs, keep airways clear — especially during illnesses — and assist with breathing at night, when breathing is shallower and may not deliver enough oxygen. Noninvasive devices can be removed or discontinued to allow for eating, drinking and talking.

Some of the tools and techniques your care team might suggest include:

  • A resuscitator bag and mask. This device can help strengthen the lungs. You place the mask over your child's mouth and nose and squeeze the other end to deliver air as your child breathes in.
  • Cough-assist device (insufflator-exsufflator). You place a mask over your child's mouth and nose. A tube connects the mask to a machine. You set the machine to deliver a certain amount of pressure as your child breathes in, helping to expand the lungs more fully. This helps strengthen the lungs. The device can also be set to suck out at a certain pressure level. This can help your child cough more effectively.
  • Manual techniques. Your care team can teach you to use your hands to apply pressure in certain areas to help your child cough. This helps the child clear mucus from the lungs, which you can then suction out.

    You can also use your hand or a vibrating device or vest on your child's chest to help loosen mucus in the lungs. This makes the mucus easier to cough up.

  • Bilevel positive airway pressure (BiPAP). This device delivers air pressure through a mask placed over the mouth, nose or both. The pressure increases to hold the airway open and deliver a higher volume of air when breathing in and lowers when breathing out. BiPAP can be used at any time of day, but it's often used during sleep, when breathing may be shallower.
  • Mechanical ventilator. Although ventilators can be used in more-invasive ways, they also can be used like BiPAP. They can be set to deliver air from the environment, without added oxygen, through a mask during sleep or when awake.
  • Sipper ventilator. This device is attached to a wheelchair and allows a person to take a quick sip of air pressure from a mouthpiece. The short burst of air can be helpful when needing to talk and breathe at the same time.

Noninvasive devices and methods may not be enough when symptoms are severe.

Invasive breathing support

When symptoms are more severe, more-invasive breathing support methods are an option to discuss with your doctor. A machine called a mechanical ventilator can be programmed to deliver air through a tube. The tube is inserted down the windpipe through the mouth or into a surgical hole in the windpipe and neck (tracheostomy).

After some time to adjust, it's possible to eat, drink and talk with a tracheostomy tube.

Discuss options with your care team often

Breathing support needs may change as spinal muscular atrophy progresses and during times of illness. Talk to your care team regularly — every three to six months — about what options are best for you.

Sept. 23, 2020