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Good Night, Sleep Tight

Best practices for PAP therapy in children with sleep-disordered breathing

Vol. 19 • Issue 9 • Page 24

Pediatric OSA Treatment

The use of noninvasive positive airway pressure for management of sleep-disordered breathing is increasing among children. This includes obstructive sleep apnea, but also chronic respiratory insufficiency in children with obesity or neuromuscular weakness.

Megan, for example, is an 18-year-old with Down syndrome who has been treated for OSA at the pediatric sleep disorders center, Children's Hospital of Wisconsin, Milwaukee. Her story highlights several key points related to pediatric patients' care. Children are not just small adults, and it is important to identify the appropriate system and mask interface. Megan and her family required extensive support and education during the initiation of PAP therapy. She had difficulty acclimating to the mask interface and required frequent monitoring during the desensitization period.

While children with Down syndrome are at higher risk for OSA due to craniofacial anomalies, the incidence of OSA in all children is estimated at 3 percent. The most common cause of OSA in children is adenotonsillar hypertrophy, and adenotonsillectomy is the primary treatment; however, PAP therapy may be indicated as follows:

• when adenotonsillectomy is not indicated or contraindicated

• when adenotonsillectomy fails to resolves symptoms, usually children with additional risk factors (obesity or craniofacial anomalies, for instance)

• prior to adenotonsillectomy in children with severe sleep apnea

• as a means of noninvasive ventilatory support in children with hypoventilation secondary to neuromuscular weakness or central sleep apnea secondary to a brain abnormality.

PAP systems

PAP therapy typically is initiated in school-age children or adolescents. The Food and Drug Administration has approved use of PAP for children ages 7 and older who weigh more than 40 pounds. But there are reports of children as young as 2 years old managed with PAP therapy. A physician's letter of medical necessity in combination with sleep study results usually is adequate to garner insurance coverage.

Several PAP modes are available for children. Continuous positive airway pressure is the mainstay of therapy for OSA management. The American Academy of Sleep Medicine 2008 guidelines recommend for children younger than 12 years old, the minimum pressure should be 4 cm H2O, with a maximum of 15 cm H2O. For children older than 12 years old, the recommended maximum pressure is 20 cm H2O.

Bilevel positive airway pressure therapy is used if the child is intolerant of higher CPAP pressures or if there are continued respiratory events at the maximum recommended pressure. Bilevel therapy can be used as a means of noninvasive ventilatory support for children with hypoventilation secondary to obesity or neuromuscular weakness. According to AASM guidelines, the minimum starting expiratory positive airway pressure (EPAP) is 4 cm H2O, with a minimum inspiratory positive airway pressure (IPAP) of 8 cm H2O. The recommended maximum IPAP is 20 cm H2O for children under 12 years old and a maximum of 30 cm H2O for children over 12 years old. An excess of 30 cm H2O of upper airway pressure increases the risk for barotrauma and other morbidities.

A backup rate is added to bilevel therapy when central apneas are observed. Of note, children with hypoventilation secondary to neuromuscular weakness may be prone to intermittent central apneas and usually benefit from a backup rate.

A ramp feature may be helpful for children who are unable to fall asleep with the PAP in place. The ramp is set for 10 to 20 minutes, and the child falls asleep as the PAP ramps up to the final setting.

PAP interfaces

It is important to select an appropriate interface that fits a child well and stays secure. Only a few interfaces are FDA-approved for children. Sometimes the smallest size of an adult interface can be used, although it may be necessary to modify the headgear to fit a child's smaller head.

Only one full face mask is designed specifically for children. Some children cannot take off the full face mask by themselves and have significant risk for aspiration if they vomit. Consider an alternate interface and chin strap if the child is prone to mouth breathing.

In some cases, it may be necessary to try a variety of mask interfaces. For example, when a child is unable to tolerate a nasal mask due to skin breakdown, claustrophobia, or other issues, nasal pillows or a nasal cannula device may be selected.

Retrusion of the maxilla or upper teeth is a concern that has developed as more children and younger children are being started on PAP therapy. The hypothesis is that pressure from the mask, even a properly fitted mask, can causes displacement of the teeth, in some cases causing a significant underbite. The mask interface may need to be adjusted or nasal pillows considered. Dentists should be made aware if a child is being treated with PAP therapy.

Getting started

Compliance with PAP therapy can be improved with patient and family education and support. Often this starts once a child has a diagnostic sleep study to confirm the presence and severity of sleep-disordered breathing; PAP therapy is initiated during a separate study. In the interim, the child is fitted with a mask interface and tries out the mask at home gradually.

Initially, the child wears the mask during periods of wakefulness such as while being distracted by watching a TV show. Eventually, the child is able to fall asleep with the mask on, and the parents remove it once the child is asleep.

Success during this desensitization period often is based on the parents' ability to remain calm and persistent. They can encourage their child to pretend to be a jet pilot or scuba diver, or have their child put the mask on a teddy bear or doll. In Megan's case, positive rewards were helpful, including a favorite breakfast of French toast if she wore the mask at night.

Maintaining compliance

Using PAP therapy on a consistent basis takes commitment from the child and family. A sleep center staff member with expertise in family-centered care can give them tips to improve PAP compliance and address common complaints.

Eye irritation can be corrected by reseating the mask on the face and readjusting the headgear; the mask should be as loose as possible while still creating a seal.

Skin irritation occurs if the mask does not fit properly or if it becomes dirty or worn out. The mask should be washed daily and then air-dried. Most insurance companies allow for a mask replacement every six months. Vitamin E cream can be useful to heal skin irritation.

Children using PAP therapy may complain of a dry mouth upon awakening in the morning. Nasal congestion and nosebleeds also may occur because of breathing in dry air. This usually is relieved by adding humidity (either heated or cool) into the PAP system. The amount and temperature of the humidity depends on the time of year and ambient temperature. A nasal saline spray at bedtime and in the morning may be helpful.

For children with allergic rhinitis, it is especially important that the PAP machine is placed on the bedside table rather than the floor to avoid dust being pulled into the system.

Children using PAP therapy can be managed successfully over a long term. They should be followed in an outpatient setting at least twice yearly. Retitration sleep studies are ordered periodically, especially if the child has experienced a significant change in weight.

Our pediatric sleep disorders center managed Megan's care for several years, and we paid close attention to her overall growth and development with appropriate interventions as necessary. She soon will be transitioning to sleep specialist who follows adult patients.

Korina Flint, RRT, is a noninvasive specialist at the pediatric sleep disorders center at Children's Hospital of Wisconsin, Milwaukee. Lynn A. D'Andrea, MD, is the sleep center's medical director.


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