Obstructive sleep apnea (OSA) is prevalent in 1% to 4% of the general pediatric population.1 The first line of treatment for OSA in this group is usually adenotonsillectomy (AT). In those for whom AT is not an option, or has been unsuccessful in eliminating the OSA, the next line of therapy is positive airway pressure (PAP), either continuous (CPAP) or bilevel. Achieving good compliance with PAP on a regular basis, especially in children with developmental delay and/or special needs, is often seen as quite daunting. Convincing otherwise healthy adults with OSA to buy into the use of PAP on a regular basis is not always easy2; this can be magnified several-fold in children who, because of their developmental stage, are unable to comprehend the reasons for using it, are unwilling to cooperate with its use, or encounter sensory integration issues with the therapy. Though good compliance with PAP can be difficult to achieve in children, with time, determination, and the right combination of tactics, the likelihood of success can be increased.
O’Donnell et al3 reported on 79 children ages 6 to 18 started on nasal CPAP during a 46-month period. In this series, the child and at least one caregiver met with a sleep technologist, in advance of the titration study, who selected a suitable mask, instructed the parents on how to gradually apply the mask, and, in some cases, recommended the use of low pressure untitrated PAP at home in advance of the titration study to accustom the child to the sensation of airflow on the face. Eighteen percent (14/79) refused to use PAP; of those who did use it, 48% (31/65) took to it immediately. The other 52% of children (34/65) gradually adapted to its use over a period of time ranging between 9 and 295 days. Adherence was greatest in children ages 6-12 compared with those under 6 or between the ages of 13 and 18. The average daily use in all children was 4.7 hours/night.
While using PAP in children can be challenging, it is often perceived as more difficult than it is. This perception can result in undertreatment when its use is discontinued after providers and families “give up,” assuming there is nothing that can be done to make its use easier. It can also lead to overtreatment, when other, more aggressive interventions are chosen without PAP having been given a proper chance to succeed.
THE RIGHT COMBINATION
The Children’s Hospital Boston Sleep Disorders Program works to enhance adherence with PAP in the pediatric population. By being “compliance minded” from the introduction of the therapy to applying close monitoring, intervention, and follow-up strategies, pediatric CPAP patients can have a better chance at successful therapy. The following protocol is employed at the Children’s Hospital Boston Sleep Disorders Program.
MASK FITTING AND HABITUATION
Once it has been determined that a child needs PAP, an assessment is made as to whether a preliminary mask fitting independent of the titration study itself is necessary. While the default is to have a mask fitting in advance of the titration, some of the older children or adolescents who do not have underlying developmental, medical, or psychiatric disabilities are brought into the sleep lab for a mask fitting on the night of the study itself.
The mask fittings are done by dedicated sleep technologists, and last between 30 and 60 minutes. An appropriate size mask is chosen, and in a process resembling a game, the mask is gradually brought to the child’s face and withdrawn in a nonthreatening way until it can be successfully applied and the technologist is able to determine that it fits properly. The parent is taught how to apply the mask and the headgear, as is the child when they are developmentally capable of doing so.
If the child appears to be comfortable wearing the mask, is able to express this, and has no apparent difficulties or anxiety, the titration study is scheduled at the end of the mask fitting. However, when the child is younger or developmentally delayed, the parent is given the mask to take home and is instructed to let the child play with it, and to put it on the child’s face while they are watching television, being read to, or getting into bed for a cuddle before going to sleep. The intention is to create positive associations with wearing the mask. The parent is asked to schedule the titration once the child has habituated to the mask (a process that can happen almost immediately or take several weeks).
This is similar to some of the measures described by Kirk and O’Donnell4 to facilitate the use of PAP and the PAP interface. These included: placing the mask within a toy box; employing positive reinforcement behavioral techniques; gradually applying the mask and headgear during wakefulness and while the child was falling asleep; and the use of untitrated CPAP at low pressure settings. They also noted their policy of proceeding to a formal titration study only when a child was able to sleep for 3 or more hours with the mask on their face, demonstrating tolerance of it.
DURING THE TITRATION STUDY
PAP is initiated in accordance with the comfort level of the child. Some children, though willing to wear the mask at home, refuse to wear it the night of the titration. If this is the case, placement of the mask is deferred until after the child has fallen asleep. Other children will agree to wear the mask, but do not tolerate the PAP itself, in which case the PAP is only started after the child has fallen asleep.
PAP (generally CPAP) is titrated to eliminate obstruction in 1-2 cm H2O increments, and in cases in which the level of CPAP necessary to control obstruction is seen to cause expiratory discomfort, bilevel is initiated so as to afford expiratory relief and make its use more tolerable.
FOLLOWING THE TITRATION
PAP is ordered and a follow-up clinic appointment is scheduled for 2 to 3 weeks after the titration study. This time frame is intended to allow sufficient time for the study to be interpreted, the order placed, insurance approval obtained, and the PAP to be in use for at least 1 week prior to the follow-up visit. The family is instructed to bring the mask and the machine to the clinic visit, and all aspects of its use are addressed. As studies have shown that up to 50% of adults discontinue CPAP soon after starting to use it,5 identifying and addressing those issues that may prevent its successful use in a timely fashion is very important so as not to allow them to impede its use.
If skin irritation and/or breakdown is noted (which can be especially problematic in infants because of their sensitive skin), the use of a protective barrier or even alternating between two masks that apply the pressure necessary to create a seal over the face differently may be considered.
The mask is applied to the child’s face, the machine is turned on, and the settings, including those of the ramp and the humidifier, are checked. The examiner typically runs his fingers around the perimeter of the mask, checking for evidence of air leak, particularly over the nasal bridge, which may result in the jetting of air into the eyes, and can be unrecognized in nonverbal children. The presence of an air leak that is not easily corrected by adjusting the headgear or its position is an indication to change to a different mask. The importance of identifying a poorly fitting mask cannot be overstated, and as most of the manufacturers have a 30-day replacement policy, doing so within this time frame can save the family significant expense. Upon removing the mask from the child, erythema at the contact points with the face can indicate a need to adjust the headgear and make it looser while still achieving a good seal.
Complaints of waking up with a dry mouth, or breathing through an open mouth, while using a nasal mask may be the result of nasal congestion and insufficient use of the heated humidifier. The parent and child (if capable) are encouraged to increase the humidifier settings as high as tolerated to reduce nasal mucosal engorgement and the increased resistance it can cause. One should also weigh whether the child might benefit from the use of a chin strap or a full face mask.
One problem that can result from the use of the heated humidifier is the condensation of water in the air hose, which then sloshes around, disrupting the child’s sleep. The use of an insulating hose cover can prevent this.
Some children with swallowing dysfunction have a tendency toward aerophagia, resulting in bloating and concerns about possible emesis. As many of these children have a G tube, venting it can relieve and prevent gastric distention. If the child receives nighttime feeds through the G tube, adjustments in the feeding schedule such as lengthening the feeding period and reducing the rate may be necessary.
When a child has profound developmental delay and cannot tolerate the mask, the use of a hypnotic may be helpful, allowing the PAP to be started once the child has fallen asleep. When a child finds using the PAP at the prescribed pressure settings uncomfortable, it may be necessary to turn it on after they have fallen asleep; maximize the ramp time; lower the pressure to allow for gradual habituation over time; and/or transition to bilevel.
Downloaded compliance data can be very useful for the treating physician. Research has found that parents significantly overreport the hours PAP is being used by their children. Marcus et al6 reported on 29 children ages 2 to 16 followed for 6 months after being started on PAP to treat OSA. Prior to its initiation, the children underwent 2 weeks of mask habituation while awake through behavioral techniques taught to the parents. Compliance data for the first 6 months of use were downloaded from the devices and analyzed. Eight children dropped out of the study; the data for the other 21 showed an average nightly use of 5.3 hours/night. Of particular interest was the fact that parents overreported their child’s use of PAP by more than 30% on average as compared to the actual data.
However, it is not unusual for parents to underestimate the actual use, to become frustrated by what they perceive as a lack of progress by their child in adapting to the PAP, and to become discouraged, even to the point of giving up on it. As the adaptation can be a lengthy process, being able to demonstrate to the parents that their child is indeed making slow but steady progress despite their impression that their child keeps on removing the mask at night can be very reassuring and sustain their efforts to achieve consistent use.
Once regular use is achieved, follow-up visits are scheduled in accordance with the child’s age and circumstances. In addition to the points discussed previously, attention is paid to whether the child has outgrown the particular interface prescribed, and whether replacement disposables are being supplied on a regular basis. Also, it is important to consider any changes in the child’s medical status (growth, changes in body weight, recent surgeries, or medication changes) that may have rendered the PAP settings inappropriately low or high, and that would be a reason for repeating a titration study.
- Lumeng JC, Chervin RD. Epidemiology of pediatric obstructive sleep apnea. Proc Am Thorac Soc. 2008;5:242-252
- Merrell JA, Shott SR. OSAS in Down syndrome: T&A versus T&A plus lateral pharyngoplasty. Int J Pediatr Otorhinolaryngol. 2007;71:1197-203.
- O’Donnell AR, Bjornson CL, Bohn SG, Kirk VG. Compliance rates in children using noninvasive continuous positive airway pressure. Sleep. 2006;29:651-8.
- Kirk VG, O’Donnell AR. Continuous positive airway pressure for children: a discussion on how to maximize compliance. Sleep Med Rev. 2006;10:119-27.
- Engleman HM, Wild MR. Improving CPAP use by patients with the sleep apnoea/hypopnoea syndrome (SAHS). Sleep Medicine Review. 2001;7:81-99.
- Marcus CL, Rosen G, Ward SL, et al. Adherence to and effectiveness of positive airway pressure therapy in children with obstructive sleep apnea. Pediatrics. 2006;117:e442-51.
Dennis Rosen, MD, is a pediatric pulmonologist and sleep specialist at Children’s Hospital Boston, where he is a member of the Division of Respiratory Diseases and associate medical director of the Sleep Disorders Program. He is an instructor in pediatrics at Harvard Medical School. The author can be reached at firstname.lastname@example.org.