CPAP and Bilevel PAP
Non-Invasive Ventilation in Children
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.
- 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)
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)
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
- 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.
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.
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
- 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)
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.
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.
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.
Resources
Information & Support
For Parents and Patients
American Sleep Apnea Association
A nonprofit 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)
Information for families that includes description, frequency, causes, inheritance, other names, and additional resources;
from the National Library of Medicine.
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 (4) (show) | | MT | NM | NV | UT |
|---|---|---|---|---|---|---|---|---|---|
| Pediatric Otolaryngology | 6 | 1 | 5 | 1 | 5 | 9 | |||
| Pediatric Sleep Medicine | 2 | 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)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.
Sleep Apnea in Children (clinicaltrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.
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
Page Bibliography
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