Cystic fibrosis (cystic fibrosis, CF) is one of the most common autosomal recessive life-limiting genetic disorders in the Caucasian population. NIV is a critical disease management strategy, but when is it most effective and when should we use it?
This study is a retrospective analysis of data prospectively collected in the electronic records of the Leeds Cystic Fibrosis Center. Data were included if the patient was 17 years of age or older, had confirmed a diagnosis of CF, and had received 12 hours or more of NIV between January 2008 and December 2018. Excluded patients were those with a transplant or with OSA. Results and indication for NIV were analyzed. Total n = 56.
What is non-invasive ventilation?
In short, non-invasive ventilation (NIV) is the delivery of oxygen through a face mask, which eliminates the need for an endotracheal airway. NIV achieves comparative physiological advantages over conventional mechanical ventilation by reducing the work of breathing and improving gas exchange.
NIV creates a positive airway pressure – the pressure outside the lungs is greater than the pressure inside the lungs. This causes air to be forced into the lungs (down the pressure gradient), reducing the effort required for breathing and reducing the work of breathing. It also helps expand the chest and lungs by increasing the residual functional capacity (the amount of air remaining in the lungs after an exhalation) after a normal (tidal) exhalation. This is the air that is available in the alveoli for gas exchange. There are two types of NIV; non-invasive positive pressure (NIPPV) and negative pressure ventilation (NPV).
Results
T2RF was the most common indication for onset of NIV, followed by acute hypercapnic respiratory failure.
The most common side effects of starting NIV were dizziness / lightheadedness and morning headache.
The mean initial inspiratory pressure was 14 cm H2O and increased to 17, while the mean exp pressure was 5 cm H2O.
The average oxygen demand was 2 liters per minute to maintain target saturation.
Commercially available face masks were overwhelmingly preferred.
~ 10% of patients in the center required NIV for a period of 10 years.
4 participants had a pneumothorax.
The liability is ~ 40-50%.
The results of this retrospective analysis are in agreement with the data collected by the UK CF registry between 2007 and 2015. There is currently insufficient data to clearly define clear criteria for starting treatment, as there are few RCTs of NIV in CF. The current justification for use is based on other criteria for long-term conditions such as COPD.
The majority of people with CF who received NIV were on the waiting list for transplants or were being considered for a lung transplant. NIV remains a successful means of connecting CF patients to lung transplants, since
its long-term use helps to stabilize lung function. NIV remains the mainstay in symptomatic care for patients receiving end-of-life treatment.
Clinical implications
NIV is used as an additional therapy for the treatment of advanced CF lung diseases in a similar way to other chronic respiratory diseases. Subjects who continued treatment with NIV showed a stabilization of lung function despite the same frequency of exacerbations and intravenous antibiotic consumption
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