What Is a Tracheostomy?

Weak chest muscles can make breathing difficult for children with spinal muscular atrophy (SMA). This is because their lungs may not fully develop. They also have a higher risk of blocked airways and lung infections. This can cause shallow or slow breathing (hypoventilation) when the lungs cannot take in enough oxygen. Hypoventilation during sleep is an early sign of breathing difficulty.¹

Breathing support (ventilation) can help get children enough oxygen. Breathing support methods vary for each person with SMA. Most children with type 1 or 2 SMA need breathing support through a face mask (non-invasive ventilation) during sleep or when sick. Some need it more often.²

Sometimes, this does not provide enough breathing support. Breathing tubes (invasive ventilation) may be needed to get air directly to the lungs. The most common method used in SMA is a tube through a surgical hole in the neck (a tracheostomy). The surgery is generally safe, but undergoing it can be a complicated decision.²

What is a tracheostomy?

A tracheostomy is a small surgical hole in the neck for a breathing tube. The surgical procedure to create the hole is called a tracheotomy.³

The breathing tube is called a tracheostomy tube or “trach.” The tube is connected to a ventilator to get air directly to the lungs. This can support breathing long-term when a medical condition or emergency causes severe breathing difficulty.³

If it is no longer needed, the hole can heal shut or be surgically closed. But for many people, a tracheostomy is permanent.

When do people with SMA need a tracheostomy?

When non-invasive ventilation does not provide enough support, invasive ventilation may be needed. This refers to devices used inside the body, such as a breathing tube.¹

Breathing tubes may be needed short-term, like during a cold or after surgery. They may also be needed long-term if non-invasive techniques are not enough after recovery. There are 2 commonly used invasive ventilation techniques used in SMA:¹

• Endotracheal tube – A breathing tube through the mouth, which can be used for a few days or weeks before irritating the mouth and throat
• Tracheostomy tube (trach) – A breathing tube through a small surgical hole in the neck, which can be used for longer

Tracheostomy ventilation is most often needed in children with type 1 SMA. Children with type 2 SMA often need non-invasive ventilation. They rarely need a tracheostomy. People with type 3 or 4 SMA do not need tracheostomy ventilation.²

What can I expect during a tracheotomy?

Most tracheostomies are performed in an operating room under general anesthesia. This makes the child unaware of the procedure. If doctors are worried that general anesthesia will cause breathing problems, they will numb only the neck and throat.³

During surgery, doctors make a small cut through the skin at the front of the neck. They will pull back surrounding muscles to expose the windpipe (trachea). Doctors will create the tracheostomy hole on the windpipe. They will insert a tube with a strap to keep it from slipping out of the hole.³

After the procedure, your child may stay in the hospital for a few days to heal. Bring comfortable clothing, personal care items, and entertainment. During this time, doctors will teach caregivers certain skills. This includes how to eat, care for the tube, and cope with dry air.³

What are the risks and concerns of a tracheostomy?

Tracheostomies are usually very safe. Some possible immediate complications include:³

• Bleeding
• Damage to the windpipe
• Temporary breathing problems or pain because of air buildup
• Misplacement of the tube

Long-term complications are more likely if the tube is in place for a long time. These problems include:³

• Blockage or movement of the tube
• Life-threatening bleeding
• Infection of the trachea or lungs

Your doctor will regularly monitor your child for complications. They can also give instructions about when to call them about problems.

Aside from medical risks, the decision to undergo a tracheostomy can be difficult and complicated. Every family has different beliefs and priorities. Some families seek to extend life as long as possible. Others want just enough care to reduce illness and promote comfort. There is no correct or best decision for every child.¹

Ask your doctor early about all respiratory care options, as well as palliative (comfort-focused) care. Tell them about your values. Doctors can discuss options based on the child’s clinical status, prognosis, and quality of life. They can also help create a plan for when different situations arise. Even if the plan changes in the future, this can help you and the care team act quickly in an emergency.¹