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Airway Clearance Therapies

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Positive Pressure Overview

Some kids with chILD need more than just oxygen to help them breathe. The lungs might not be able to bring in enough oxygen or get rid of carbon dioxide. These children may need a machine that delivers air with pressure into their lungs. This is called positive pressure ventilation or positive pressure breathing. 

There are different types of positive pressure breathing. When the pressure is given through a mask or nose tube, it is called non-invasive. Some children need pressure to be given through a tube in the neck. This positive pressure is called invasive.

Non-Invasive Positive Pressure (CPAP/BiPAP)

CPAP and BiPAP machines can give breathing pressure using a face or nose mask.

  • CPAP stands for Continuous Positive Airway Pressure.  With CPAP, the machine gives 1 pressure to the lungs all of the time. The amount of pressure can change based on what the child needs. Extra oxygen can also be given through the machine.
  • BiPAP stands for BiLevel Positive Airway Pressure.  With BiPAP, the machine gives 2 different pressures. The pressure is higher when the child breathes in and lower when the child breathes out. The amount of pressure can change based on what the child needs. Extra oxygen can also be given through the machine. BiPAP machines can also have a rate to make sure the child is not breathing too slowly. 

Some children only use CPAP or BiPAP during sleep.  Other children may need CPAP or BiPAP for time during the day and night. 

Invasive Positive Pressure (Tracheostomy/Ventilators)


A tracheostomy is a short breathing tube.  It goes from the outside of the neck, through the skin and to the inside of the trachea (windpipe). The tube stays just below the voice box or vocal cords. A surgery is needed to place the tracheostomy. This is done by a pediatric surgeon or an ear, nose, and throat (ENT or otolaryngology) doctor.

Tracheostomies can help if a child needs positive pressure for most of the day and night. Other kids may need a tracheostomy if they have blockage in their airway.

If your child needs a tracheostomy, their care team will talk about the details of the surgery and how life will be different. This can be scary for families. But it may be the best way for your child to breathe. 

Click here for more tracheostomy information from the American Thoracic Society.


Ventilators are machines that give positive pressure to help children breathe. They can give just a little bit of help (like just CPAP or BiPAP). Or they can do all of the breathing for the child. Sometimes they are used for non-invasive pressure. Sometimes they are used for invasive pressure.

Ventilators are used in the hospital but smaller machines can be used at home. Your child’s care team can help decide how much time they spend on the ventilator. Some children need the ventilator all of the time. Some children can come off of the ventilator as they grow older.

A child on a ventilator can never be left alone. Your child’s medical team will help train you in how to take care of the ventilator. When possible, they can help with nursing support at home. You will have a durable medical equipment (DME) company to give you the supplies and help you when problems come up at home. Click here for more information on ventilators from the American Thoracic Society.


An echocardiogram (ECHO) is an ultrasound of the heart. This test is used to see the structure of the heart and measure how well it is working. Since problems with the heart can look like chILD, it is common to need an ECHO at some point during diagnosis. Problems with the lungs can make lung blood vessels and parts of the heart work harder than they should. This can lead to high blood pressure in the lungs, called pulmonary hypertension. An ECHO is used to watch for signs of pulmonary hypertension. 

Getting an ECHO does not hurt. Your child will need to stay very still during the test. There is no radiation involved. 

Lung Transplant

What is lung transplantation?

A lung transplant is a surgery where unhealthy lungs are removed and replaced with healthy lungs. The new lungs come from a person who has donated their organs after death. Lung transplants are used when lung disease is very serious and no new medicines or procedures will allow for improvement . Some children with chILD may be sick enough to need a transplant. 

Getting a lung transplant is not easy or simple. The choice is complex and different for every family. Lung transplant is not a cure. It is a treatment option that can allow patients to live longer and live better. It is important to know that you are trading one set of problems for new challenges after transplant. Children will need a lifetime of medicines and close follow-up care by their lung transplant team. 

Who needs a lung transplant?

Lung transplant is not for everyone. It is a shared decision between your family, your lung doctors, and the lung transplant team. If you are thinking about lung transplant, it is best to meet with a transplant team early so choices can be made before transplant becomes an emergency.

Some lung diseases that may need a lung transplant:

  1. Cystic fibrosis
  2. Interstitial lung disease
  3. Surfactant protein dysfunction
  4. Alveolar capillary dysplasia
  5. Bronchiolitis obliterans
  6. Pulmonary hypertension (high blood pressure in the lungs)
  7. Heart disease or heart defects affecting the lungs or lung blood vessels
  8. Bronchopulmonary dysplasia (BPD)
  9. Pulmonary fibrosis (scarring)

Basics of lung transplant:

A lung transplant team will follow your child closely before, during, and after lung transplantation.

The transplant evaluation

There are many tests that need done before lung transplant. These tests help the team decide if a lung transplant will help your child. They also help decide the best time for a transplant. Some children can wait years before a lung transplant is truly needed. These tests may include blood work, heart testing, chest imaging, pulmonary function testing, procedures, and meeting with other doctor teams. 

The transplant team will also have you meet with a social worker to make sure the child has enough family support to take care of them after transplant. If the transplant center is far away, your family may have to move closer to that city to live before and after transplant. Your family will be taught about lung transplant to help you decide if it is the best treatment for your child. 

The waiting list

After the choice is made for lung transplant, your child will be placed on the transplant waiting list. The United Network for Organ Sharing (UNOS) is the group that controls organ transplants in the United States. The wait time for lung transplant depends on how sick the child is and finding lungs that match the child. Some people wait days for a transplant, while others may stay on the waiting list for years.

Lung transplantation

When it is time for transplant, a cardiothoracic (chest) surgeon will perform the procedure. This is a big surgery that takes several hours. Your lung transplant team and surgery team will follow closely during recovery. Children are in the hospital for many weeks to months to recover and start their life with new lungs.

Life after lung transplant:

After transplant, your child will need to be on medicines to make sure their body doesn’t reject the new lungs. It is very important to take all medicines, watch for side effects, and  have regular visits with the lung transplant team for follow-up. These medicines lower the immune system. If not careful with medicines or follow-up, children may get very sick with organ rejection or infection. 

Nobody can predict how long a child will live after lung transplant. About half of the children who have a lung transplant are alive after 5 years. Hopefully those extra years are filled with easier breathing and a better life. Each child is different. If all goes well, lung transplant can give the child a longer and better life. 


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