CPAP and Bilevel PAP

Non-Invasive Ventilation in Children

CPAP (continuous positive airway pressure) and Bilevel PAP (bi-level positive airway pressure) systems deliver air via contoured nasal or full-face masks to maintain a set airway pressure. This results in increases in the functional residual capacity (the volume of air in the lungs at the end of expiration) and/or tidal volume (volume of air in each breath). These technologies are used in both acute and chronic care settings, including the home environment. Their most common use in children is for obstructive sleep apnea.

CPAP delivers a constant flow of pressurized air throughout the breathing cycle, resulting in increased mean airway pressure and functional residual capacity, but does not impact tidal volume. Bilevel PAP provides two different levels of pressure: greater pressure during inspiration (IPAP) to improve tidal volume and a lower pressure during exhalation (EPAP) to increase mean airway pressure and functional residual capacity. Bilevel PAP can also be set to provide “back-up breaths” to maintain a certain minimum number of breaths per minute.

Introducing and maintaining use of these technologies can be a challenge for children and their caregivers, in ensuring tolerance and effectiveness and minimizing side effects. Patients and their families should expect the need for high bedside caregiver presence, at least initially. One to three weeks of gradual ‘desensitization’ to get used to breathing with the machine is often required. Rarely, sedation is needed to help children tolerate the equipment.

Indications

CPAP or Bilevel PAP may be offered to children whose respiratory systems are compromised by weakened respiratory muscles, weakened respiratory drive, airway obstruction, injury to lung tissue, or underdeveloped lungs. For patients with chronic respiratory problems, they are usually used for 6 to 10 hours at night. In acute settings, they may be used as an alternative to endotracheal intubation or in the weaning process from mechanical ventilation.

Specific indications for use of CPAP/Bilevel PAP include:

Hypoventilation and hypercarbia in patients with progressive neuromuscular and restrictive chest wall disorders, such as muscular dystrophy, spinal muscular atrophy, morbid obesity, and kyphoscoliosis
Chronic obstructive airway disease (COAD), such as cystic fibrosis
Obstructive sleep apnea (OSA), characterized by symptoms ranging from snoring with mild hypoxemia to prolonged episodes of obstructive hypoventilation with hypercarbia
Impending respiratory muscle fatigue usually as part of acute respiratory illnesses
Asthma
Bronchiolitis
Pneumonia
Possible use for children with central hypoventilation syndrome (CHS); children with CHS demonstrate abnormal breathing patterns with symptoms of cyanosis, apnea (absence of airflow resulting in respiratory pause), and hypopnea (shallow breathing episode)

Alternatives

For some patients, alternatives to Bilevel PAP and CPAP include:

Removal of the tonsils and adenoids (adenotonsillectomy) for OSA
Weight loss for patients with obesity
Oral appliances, although data in children is limited and use of the devices can impact craniofacial growth
Airway surgery such as septoplasty (alteration of the nasal septum), nasal turbinate reduction, uvulopalatopharyngoplasty (alteration of the back of the palate and upper airway), and lingual tonsillectomy
Jaw surgery (maxillary or mandibular advancement surgery)

Equipment

CPAP-Bilevel PAP machines deliver a stream of heated, humidified, compressed air via a hose to a nasal pillow, nasal mask, full-face mask, or hybrid. This “splints” the airway (keeps it open with air pressure) so that unobstructed breathing becomes possible, reducing and/or preventing apneas (breathing stoppages) and hypopneas (shallow breathing). The masks are secured to the patient using a headband or similar attachment device.

The equipment generally includes:

Flow generator (PAP machine) provides the airflow. The machines are fairly small and quiet, weighing from 6 to 12 lbs. including cables and cords. They are portable, although most pediatric patients use them solely at home during sleep.
Hose connects the flow generator to the mask interface with the airway.
Mask (nasal, full face mask or nasal pillows) provides the connection to the user's airway. For non-invasive ventilation to be effective, a good mask fit and seal are crucial

Optional Equipment

Humidifier adds moisture and heat to inhaled air. Heated humidified air is very important for the comfortable use of CPAP/Bilevel PAP and to avoid side effects such as nose bleeds, dry mouth, voice changes, cough, and congestion
Mask liners may be used to prevent excess air leakage and to reduce skin irritation and dermatitis
Flexible chin straps may be used to help the patient avoid breathing through the mouth (full-face masks prevent this), thereby keeping a closed pressure system. The straps are elastic enough that the patient can easily open his/her mouth if necessary. Modern straps use a quick-clip instant fit. Velcro-type adjustments allow for quick size adjusting, before or after the machine is turned on.

The machines supply air at a prescribed pressure (also called the titrated pressure), usually measured in cm of H2O. Most machines supply room air but, if the patient is experiencing suboptimal oxygen levels, oxygen can be delivered into the machine or mask using a special adapter.

Care/Maintenance

As with any therapy, caregivers must be aware of potential complications and side effects. The masks may cause excoriation at the points of skin contact and acneiform rashes. Application of protective barriers, such as hydrocolloid dressings, and use of antibiotic creams may resolve these issues. Alternating between different types of masks may also limit skin problems. Eye irritation related to the mask or leaking air is comforted with lubricating drops, correcting the leak and/or increasing humidification.

Poor mask fit contributes to skin and eye irritation usually from leaks around the mask. Minor leaks are acceptable as long as they do not alter the machine’s effectiveness. If too tight, the mask may impact growth of the facial bones. A better approach for persistent leaks is to try various size masks to find the best fit.

The care and maintenance required for PAP machines varies with the type and conditions of use and are typically spelled out in an instruction manual. Manufacturers recommend that the end-user perform daily and weekly maintenance. Units must be checked regularly for wear and tear and kept clean. Most masks have a lifespan of 3-6 months, airfilters in the machine last for 1-3 months and hoses usually last for 6-12 months. Inappropriate care of the equipment will result in loss of effectiveness.

Challenges/Side Effects

Common complications/side effects include:

Claustrophobia
Eye irritation
Skin irritation and sores over the bridge of the nose
Nasal congestion and sore or dry mouth
Noise that interferes with sleep (usually a sign of excessive air leak)
Nosebleeds
Upper respiratory infections
Aspiration in children with impaired swallow function

Strategies for Successful Use of Bilevel PAP in Children

Consultation with a respiratory therapist or polysomnography technician to find the correct mask and strategies for adjusting to the equipment is recommended.
Select a comfortable mask or proper size. It should be neither too tight nor too loose, and it should not leak air when attached to the hose and compressor.
Monitor and make appropriate adjustments in fit and CPAP/Bilevel PAP settings.
Use of protective barriers for skin may be considered if there is skin irritation.
Use of humidification, nasal hydration drops (e.g. saline or xylitol nasal drops)

Follow-Up and Evaluation

Patients using nightly CPAP or Bilevel PAP should be seen by their health care provider regularly. Initial follow-ups may be as frequent as 1-2 times per month. Once the patient has adjusted to therapy, annual follow-up may suffice. [Epstein: 2009] Rationale for this is based on the fact that children grow and this results in change in airway size, pressure needs as well as severity of illness. Monitoring and addressing therapeutic side effects is also a part of the follow up evaluation. Patients with progressive neuromuscular diseases may have gradual worsening of their weakness resulting in worsening of respiratory function, swallow dysfunction and kyphoscoliosis. At some point of illness progression, patients with neuromuscular disorders may no longer be suitable candidates for continued use of non-invasive ventilation.

Assessing Patient Response

How does the clinician assess whether CPAP-Bilevel PAP is successful? Improved daytime functioning including decreased tiredness/sleepiness as well as improved concentration and energy levels. Patients may report reduced dyspnea, and blood gas values generally improve. Agitation, increased confusion, hemodynamic instability, worsened oxygenation, recurrent pneumonia or difficulty clearing secretions would serve as indicators that PAP is not effective.

Cost/Coverage

Total costs to purchase CPAP-Bilevel PAP systems are estimated at $1,000 to $5,000. Patients can also rent the equipment. Many major insurers, including Medicaid and Medicare, cover the cost of the technology for a range of diagnoses. Clinicians and patients are urged to contact the patient’s insurer to determine their specific guidelines. Although there is sufficient medical literature to support therapeutic effectiveness, FDA approval of pediatric use of home CPAP-Bilevel PAP equipment is limited. Home care companies, in compliance with such guidelines, may refuse to cover certain patients, resulting in difficulty with patient access to care.

Images of CPAP masks and devices

Resources

Information & Support

For Parents and Patients

American Sleep Apnea Association
A non-profit organization that promotes awareness of sleep apnea, works for continuing improvements in treatments for this serious disorder, and advocates for the interests of sleep apnea patients.

Nasal CPAP (MedlinePlus)
Brief general article about using nasal CPAP, from the National Library of Medicine's MedlinePlus

Choosing a PAP Machine (American Sleep Apnea Association)
Information about several PAP manufacturers that offer different types of machines with different features.

Obstructive Sleep Apnea (KidsHealth)
Article for parents/consumers on sleep apnea from Nemours Foundation.

Avoiding 10 Common Problems with CPAP Machines (Mayo Clinic)
Advice on dealing with common problems associated with CPAP.

Getting a Diagnosis (American Sleep Apnea Association)
Information about getting an evaluation for sleep apnea.

When Things Go Wrong with CPAP (American Sleep Apnea Association)
Information about mask discomfort, nasal congestion, headache, ear pressure, and more.

Choosing a Mask (American Sleep Apnea Association)
Information about types of masks and alternative masks.

Services for Patients & Families in Rhode Island (RI)

Service Categories	# of providers* in:	RI	NW	Other states (5)  (show)		ID	MT	NM	NV	UT
Pediatric Otolaryngology		6	1		4	5	1	5	10
Pediatric Sleep Medicine		1			3	4			3

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Studies

PAP in Children (clinicaltrials.gov)
An up to date listing of studies in these forms of non-invasive ventilation.

Sleep Apnea in Children (clinicaltrials.gov)

Helpful Articles

Marcus CL, Rosen G, Ward SL, Halbower AC, Sterni L, Lutz J, Stading PJ, Bolduc D, Gordon N.
Adherence to and effectiveness of positive airway pressure therapy in children with obstructive sleep apnea.
Pediatrics. 2006;117(3):e442-51. PubMed abstract

Hsiao KH, Nixon GM.
The effect of treatment of obstructive sleep apnea on quality of life in children with cerebral palsy.
Res Dev Disabil. 2008;29(2):133-40. PubMed abstract

Uong EC, Epperson M, Bathon SA, Jeffe DB.
Adherence to nasal positive airway pressure therapy among school-aged children and adolescents with obstructive sleep apnea syndrome.
Pediatrics. 2007;120(5):e1203-11. PubMed abstract

Praud JP, Dorion D.
Obstructive sleep disordered breathing in children: beyond adenotonsillectomy.
Pediatr Pulmonol. 2008;43(9):837-43. PubMed abstract

O'Neill N.
Improving ventilation in children using bilevel positive airway pressure.
Pediatr Nurs. 1998;24(4):377-82. PubMed abstract

Joshi G, Tobias JD.
A five-year experience with the use of BiPAP in a pediatric intensive care unit population.
J Intensive Care Med. 2007;22(1):38-43. PubMed abstract

Pooboni SK.
Noninvasive Ventilation.
eMedicine, WebMD; (2009) http://emedicine.medscape.com/article/1417959-media. Accessed on 1/29/11.
Includes several photos of equipment and its use with patients.

Yang ML, Finkel RS.
Overview of paediatric neuromuscular disorders and related pulmonary issues: diagnostic and therapeutic considerations.
Paediatr Respir Rev. 2010;11(1):9-17. PubMed abstract

Epstein LJ, Kristo D, Strollo PJ Jr, Friedman N, Malhotra A, Patil SP, Ramar K, Rogers R, Schwab RJ, Weaver EM, Weinstein MD; Adult Obstructive Sleep Apnea Task Force of the American Academy of Sleep Medicine.
Clinical guideline for the evaluation, management and long-term care of obstructive sleep apnea in adults.
J Clin Sleep Med. 2009;5(3). PubMed abstract / Full Text

Gozal D, Kheirandish-Gozal L.
Sleep apnea in children--treatment considerations.
Paediatr Respir Rev. 2006;7 Suppl 1:S58-61. PubMed abstract

Authors & Reviewers

Initial publication: January 2010; last update/revision: February 2011

Current Authors and Reviewers:

Author:	Ameet S. Daftary, MD
Reviewer:	Nancy Murphy, MD, FAAP, FAAPMR

Authoring history

2010: first version: Matt Pacenza, MA^A

^AAuthor; ^CAContributing Author; ^SASenior Author; ^RReviewer