Review question

What are the benefits and risks of using non-invasive positive pressure ventilation (NIPPV) to treat people with cystic fibrosis (pwCF)?

Key messages

Based on current available evidence, we are not sure:

- whether one type of NIPPV is better than another;

- in which situations it helps the most;

- or for which specific purposes it works best in pwCF.

The results are also uncertain about the effects of NIPPV on mortality and quality of life. Some people found NIPPV helpful and preferred using it, while others stopped because they felt uncomfortable or could not tolerate the mask; the evidence about this is still very uncertain. Regarding safety, most studies did not report serious problems (serious adverse events). However, some described situations such as swallowing air (aerophagia) and air trapped in the lung (pneumothorax).

What is CF?

CF is a disease that is present from birth, and affects multiple body systems, especially the respiratory system. Common symptoms are persistent cough with thick mucus, frequent lung infections, and breathing difficulties. CF worsens with age, causing complications due to constant infections that damage airways, reduce lung function, and may lead to death.

What is NIPPV, and how can it assist in the treatment of pwCF?

NIPPV is a treatment where a machine is used to help the person breathe. During NIPPV therapy, the person breathes using a mask that forces the air into the lungs, keeping the airways open and requiring less effort from the individual. There are two main types of NIPPV:

• Continuous positive airway pressure (CPAP): this type of NIPPV forces air into the lungs constantly (during inhalation and exhalation).

• Bilevel positive airway pressure (BiPAP): this type of NIPPV forces air into the lungs more during inhalation and less during exhalation.

Studies suggest that NIPPV, combined with techniques to clear lung mucus, may ease breathlessness and improve oxygen levels in the blood. It can enhance sleep quality, reduce daytime sleepiness, and lessen fatigue during exercise, allowing people to exercise for more time. However, some people feel anxious and uncomfortable with a breathing mask. Most of these studies involved individuals with other lung diseases, so it remains unclear if NIPPV can help pwCF live longer or better, or if it might come with any risks for them.

What did we want to find out?

How the different types of NIPPV, used alone or combined with other treatments, can help pwCF.

What did we do?

We searched the main databases and analyzed all relevant studies available.

What did we find?

We found 14 studies with 261 children and adults at different stages of CF (including times when symptoms suddenly worsen, indicating the person is in crisis; and when symptoms remain the same as previously, meaning that the disease is stable or under control). NIPPV was compared to standard treatments, to other airway clearance methods (such as PEP mask or directed coughing), and to oxygen therapy. In some trials, it was also combined with other treatments. The duration of the treatments varied, from a single session to repeated use for up to three months.

Main results

In pwCF, the effects of BiPAP during pulmonary crises (exacerbations) are still uncertain. It is not clear whether BiPAP is better or worse than a PEP mask (a mask that creates resistance when breathing out) or guided coughing (a technique to help clear lung secretions) in terms of the amount of phlegm (thick sticky mucus) cleared and the tiredness felt by patients. The effects of BiPAP compared to oxygen therapy (air with a higher concentration of oxygen), or when used together with airway clearance techniques to shorten hospital stays, improve comfort, or reduce side effects, are also unclear.

In pwCF who are stable, the evidence is also very uncertain. We do not know whether BiPAP, compared to a PEP mask alone or in combination with other treatments, improves lung function (the amount of air the lungs can breathe in and out), sleep quality, or patient acceptance. Another device, CPAP, has also been studied in combination with airway clearance techniques (methods to loosen and clear mucus from the lungs), but the results remain uncertain.

What are the limitations of the evidence?

All studies included few participants, which may not have been sufficient to show the real effects. There are also doubts about how these studies were conducted. The confidence intervals were wide, which means that the true effect may be quite different from what appeared in the results. No study evaluated the long-term effects of the interventions, mainly focusing on immediate effects, such as one or two sessions.

Overall, we had only low to very low confidence in the results. Further larger, better‐quality trials are needed to clarify the effects of NIPPV.

How up-to-date are these findings?

The evidence is current to 2 April 2025.