Cough Assistance for Spinal Muscular Atrophy

Coughing is an important way to clear mucus and other secretions from our airways. This helps prevent the airways from getting blocked. Children with spinal muscular atrophy (SMA) have weaker chest muscles, which causes them to have a weaker cough. Without a strong cough, mucus stays in the lungs. This can decrease breathing ability and lead to respiratory infections.

Helping children with SMA cough stronger can prevent lung infections and other breathing problems. Soon after diagnosis, families should discuss airway clearance with a pulmonologist. This is a doctor who specializes in lung care. They can help create a plan of how to loosen secretions and improve coughing abilities. Children with type 1 or 2 SMA should be tested for cough effectiveness by a pulmonologist every 3 to 6 months.¹

What are some cough assistance methods used in SMA?

Coughing can be improved using a cough machine or manual cough assistance techniques. In addition, chest physiotherapy can loosen mucus to make it easier to cough out. These techniques reduce the frequency of lung infections and hospitalizations for people with SMA.^2,3

Cough machine

A cough machine pushes air into the lungs and sucks air out of the lungs through a face mask. This action helps produce a stronger cough. A common pattern is to use it for sets of 4 to 5 breaths with 1 to 2 minutes of rest in between. This machine is sometimes called an insufflator-exsufflator machine.⁴

Several brands are available, such as CoughAssist or VitalCough. Talk to your pulmonologist to find a cough machine that is right for your child. They can also advise you on what pressure and other settings to use.⁴

Manual cough assistance

In manual cough assistance, a caregiver uses hands to put gentle pressure on a child’s abdomen as they cough. This can help children exhale stronger. After manual cough assistance, mucus and other secretions can be removed from the mouth using oral suctioning. Otherwise, mucus may be swallowed. Ask your pulmonologist to show you how to perform manual cough assistance so you can do this at home.⁴

Chest physiotherapy

Chest physiotherapy is the most common way to loosen mucus that builds in the airways. This makes secretions easier to cough up. Chest physiotherapy can be done either manually (with the hands) or mechanically (with a device). With the manual technique, a caregiver uses a vibrating or clapping technique with their hands on the chest. With the mechanical technique, a vibrating device is placed on the chest.⁴

Other methods of loosening mucus can also be used, such as postural drainage. Ask your pulmonologist what technique to use and to show you how to perform each method.⁴

How are cough assistance methods used in the different types?

The exact methods to clear the airway vary for every person, depending on disease severity. Some children with SMA may need cough assistance on a daily basis. Others may only need it when they are sick. During an acute cold, children with SMA may need cough assistance more regularly. Here are common goals of cough assistance for each type of SMA:¹

Type 1 or 2 SMA

Manual chest physiotherapy and cough machines are commonly used to clear the airways of children with type 1 or 2 SMA. Oral suctioning with a mechanical pump is also an important part of airway clearance. Proactive respiratory care is especially important for children with type 1 or 2 SMA. These methods should be used proactively, depending on your doctor’s assessment of cough effectiveness.¹

Type 3 or 4 SMA

Most people with type 3 or 4 SMA have normal lung function. Your doctor should still regularly monitor cough effectiveness. If they notice a weak cough or other breathing issues, they should refer you to a pulmonologist. Otherwise, proactive cough assistance methods are usually not used.¹