Treatment
The common mainstays of management for sleep-disordered breathing
include oxygen therapy and noninvasive ventilation (NIV) such as
continuous positive airway pressure (CPAP), bilevel positive airway
pressure (BiPAP) or more advanced therapy. Oxygen therapy alone,
however, should be looked at with caution in our patient population as
it may worsen hypoventilation57. In children,
therapeutic options include surgical options such adentonsillectomy as
first line treatment for OSA. Additional options for OSA in adults
include oral devices, glossopharyngeal nerve stimulation, myofunctional
therapy and positional devices58-60. Pharmacologic
therapies for OSA, such as montelukast and nasal steroids have shown
mixed results in pediatric and adult populations, with some benefit to
montelukast being seen in children but not
adults61,62.
There are no clear guidelines or recent literature available for
treatment of isolated nocturnal hypoxemia. Although there are studies
evaluating use of oxygen therapy based on daytime hypoxemia, studies
treating based on nocturnal hypoxemia are sparse.63Multiple older studies exist recommending caution with using oxygen
therapy alone at night for treatment due to worsening
hypercarbia.45,46 In a 12-month randomized prospective
trial, Milross et al. studied the long term effects of NIV, with or
without oxygen and low flow oxygen on event free survival in adults with
CF already diagnosed with nocturnal hypoxemia by a
PSG.57 Events were defined as development of
hypoventilation, lung transplantation, or death.57They also followed for outcomes such as hospitalization, lung function,
and health-related quality of life.57 Their aim was to
determine if adults with CF and nocturnal desaturations were less likely
to develop hypercapnia on low flow oxygen or non-invasive
ventilation.57 They concluded that NIV with or without
oxygen increased event free survival compared to treatment with oxygen
alone.57 This emphasizes that CF providers need to use
caution if prescribing oxygen therapy alone.
Noninvasive positive pressure ventilation is not completely new to the
cystic fibrosis population, and has been successfully already been
incorporated in the care plan for certain patients. In stable CF
patients with awake hypercapnia, a six week trial of nocturnal
noninvasive ventilation showed improved nocturnal hypoventilation and
peak exercise capacity without improving lung function or awake
hypercapnia64. Noninvasive ventilation has also been
evaluated in cystic fibrosis patients as an adjunct to airway clearance
techniques,65,66 particularly in patients having
trouble expectorating sputum.65 The rationale is that
in patients with severe lung disease, airway clearance therapy results
in energy expenditure and use of noninvasive ventilation could allow for
decreased work of breathing and fatigue.67 In
addition, noninvasive ventilation is used pre- lung transplant, and has
been shown to slow the decline in lung function in this patient
subset65. It stands to reason that patients prescribed
noninvasive ventilation for sleep-disordered breathing may also have
additional benefits such as improved airway clearance, increased peak
exercise capacity, and a slower decline in lung function; however,
dedicated studies looking at these outcomes are necessary.