Authors: Jiaxin Tran, MD John R. Bach, MD Miguel R. Gon0alves, PhD

Pulmonary

Affiliations: From the Department of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School, University Hospital, Newark (JT, JRB); and Department of Pulmonology, University Hospital of S. Joa˜o, Faculty of Medicine, University of Porto, Porto, Portugal (MRG).

Correspondence: All correspondence and requests for reprints should be addressed to: John R. Bach, MD, Department of Physical Medicine and Rehabilitation, University Hospital B-403, 150 Bergen St, Newark, NJ 07103.

Disclosures: Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article.

0894-9115/14/9302-0182 American Journal of Physical Medicine & Rehabilitation Copyright * 2013 by Lippincott Williams & Wilkins DOI: 10.1097/PHM.0000000000000029

CASE SERIES

Alternatives to Mouthpiece Noninvasive Ventilatory Support to Permit Dental Care ABSTRACT Tran J, Bach JR, Gonçalves MR: Alternatives to mouthpiece noninvasive ventilatory support to permit dental care. Am J Phys Med Rehabil 2014;93:182Y185. Patients who are dependent on continuous noninvasive intermittent positive pressure ventilation for ventilatory support via angled mouthpiece interfaces during daytime hours often need dental interventions that are prevented by the presence of the mouthpiece. To permit dental interventions, however, the noninvasive intermittent positive pressure ventilation can most conveniently and safely be delivered via nasal interface using oximetry monitoring as long as both sedation and supplemental oxygen are avoided. Three mouthpiece continuous noninvasive intermittent positive pressure ventilation users are described who were switched to nasal noninvasive intermittent positive pressure ventilation to permit dental care. The most common mistake is to fail to cover the nasal interface exhalation portals when switching from ventilatory assistance by bilevel positive airway pressure devices with passive ventilator circuits to portable ventilators with active ventilator circuits and exhalation valves. Key Words: Dentistry, Duchenne Muscular Dystrophy, Respiratory Therapy, Neuromuscular Disease, Noninvasive Mechanical Ventilation

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espiratory muscle failure and need for up to continuous noninvasive intermittent positive pressure ventilation (NIPPV) can occur for patients with neuromuscular diseases such as Duchenne muscular dystrophy and spinal muscular atrophy,1,2 patients with high-level spinal cord injury,3 postpoliomyelitis survivors,4 and those with severe thoracic cage deformities. Whereas most such patients use ventilatory support via indwelling tracheostomy tubes, with increasing appreciation of the morbidity and mortality caused by them,5,6 more and more have turned to noninvasive alternatives.7,8 With increasingly portable ventilators, new mouthpiece ventilation-specific software (Trilogy; Philips-Respironics International Inc), invariable patient and care provider preference for noninvasive management,9 decreased morbidity and expense using it,10 and avoidance of the stigma associated with tracheostomy,6,11 it is likely that there will be more such mouthpiece NIPPV users in the future.

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Noninvasive positive pressure interfaces deliver air to the patient’s lungs via the mouth (Fig. 1), the nose (Fig. 2), the mouth and the nose, face-covering interfaces, and helmets. Typically, all of these can be used during sleep except for the 15-mm angled mouthpieces (Philips-Respironics International Inc, Murrysville, PA), which are conveniently used for daytime support (Fig. 1). Many patients with no ventilator-free breathing ability require dental interventions that are prevented by mouthpiece use for NIPPV. Other than for nasal interfaces that permit full access to the oral cavity, all other noninvasive positive pressure interfaces obstruct oral access for dental procedures.

CASE REPORTS A 65-yr-old woman with severe idiopathic kyphoscoliosis and a vital capacity of 400 ml (18% of predicted normal) who was dependent on NIPPV continuously for 11 yrs was referred to an academic dentistry service for a tooth extraction. She began using nocturnal-only bilevel positive airway pressure via a nasal interface (Confort Gel; Philips Respironics,

FIGURE 1 A 65-yr-old woman with severe idiopathic kyphoscoliosis dependent on continuous noninvasive positive pressure ventilatory support (NIPPV) for 11 yrs. During the day, the patient’s portable ventilator and accessories are on her self-designed walker that permits her to walk while using a 15-mm angled mouthpiece for NIPPV. www.ajpmr.com

FIGURE 2 The same patient as in Figure 1 receiving dental care using a nasal interface for ventilatory support.

Murrysville, PA) on a passive ventilator circuit for sleep when she was 47 yrs old and progressed to require continuous NIPPV using a volume-cycled portable ventilator with an active ventilator circuit and exhalation value via a 15-mm angled mouthpiece when awake. She walks using a walker modified to hold her respirator and mouthpiece NIPPV accessories (Fig. 1). She was switched from mouthpiece to her nocturnal nasal interface for NIPPV during the tooth extraction. However, a nasal interface for bilevel positive airway pressure requires exhalation ports to minimize CO2 rebreathing. It is a common mistake to leave the ports open when switching to using an active circuit, one with an exhalation valve, for volume-cycled mouthpiece NIPPV. This results in the ventilator-delivered air leaking out of the open ports rather than entering the patient’s lungs. Thus, the interface ports were covered to permit the dental work. Only local anesthesia was used; supplement oxygen was avoided, and oximetry was monitored. Ventilator settings of 1200-ml volumes and rate of 10 per minute, as used for mouthpiece ventilation, were unchanged for the nasal ventilation during the procedure (Fig. 2). For any dyspnea and/or decreases in oxyhemoglobin saturation (oxygen saturation as measured by pulse oximetry [SpO2]) caused by excessive oral air leakage, the dental work was paused so that she could close her mouth to eliminate oral leakage and renormalize SpO2 (Fig. 3). A 41-yr-old with Duchenne muscular dystrophy and severe scoliosis required treatment of pain from dental caries for the first time at the age of Mouthpiece NIPPV and Dental Care

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FIGURE 3 Oximetry data during the dental procedure while using nasal ventilation showing heart rate and return to normal oxyhemoglobin saturation after desaturations as the patient eliminated oral air leakage.

17 yrs then subsequently on seven occasions through the age of 37 yrs. He was wheelchair dependent since the age of 5 yrs and dependent on mouthpiece NIPPV during the day and mouthpiece/lip cover (Lipseal; Philips-Respironics International Inc) during sleep since the age of 14 yrs. He ate only pureed food but articulated speech with minimal difficulty. Below his neck, he had only slight big toe movement since the age of 17 yrs with which he operated his computer, but he could rotate his neck to grab a 15-mm angled mouthpiece for NIPPV throughout daytime hours since the age of 14 yrs. The mouthpiece was kept adjacent to his mouth by a flexible metal support arm attached to his wheelchair frame that also had a tray to hold a portable ventilator. His large carious lesions required 3 hrs of dental work on the first occasion and extensive work subsequently. He had never used a nasal interface but was switched from the mouthpiece to a continuous positive airway pressure interface (Fig. 4) (Respironics CPAP mask, Murrysville, PA) for all seven dental interventions. Because the CPAP interface was being used with an active circuit during the dental procedures, the interface ports were covered. A 30-yr-old man with Duchenne muscular dystrophy and severe scoliosis presented to a local dentist with a dental abscess. He required continuous NIPPV for which he used a 15-mm angled mouthpiece interface, which sat in the corner of his mouth throughout daytime hours,andanasalorlipcoverinterfaceforsleepfor16yrs. He had some active finger and toe movement below his neck and the ability to eat pureed foods and articulate speech. He, too, was switched to using a nasal interface, and his carious lesion and abscess were successfully treated. His SpO2 never decreased lower than 95%, and as for all three patients who leaked varying quantities of air out of the mouth, there was no distress or complications.

DISCUSSION Interestingly, whereas patient 2 was unaware of the existence of nasal interfaces, it also did not occur

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to patients 1 and 3, who were using them for sleep, to substitute them for mouthpiece NIPPV to permit dental procedures. They thought that the nasal NIPPV would not be effective because of air leakage out of the mouth. However, both passive movement of the air pushing the soft palate against the back of the tongue to seal off the oropharynx and active palatal and tongue movements likewise prevented excessive leakage during the procedures. Besides triangular nasal interface designs, nasal prongs and pillow systems could have been used. Nasal pillows consist of a pair of nasal inserts housed in a small covering that seals around the nostrils. The prongs insert directly into the nares. In addition, the patients could have been switched to using an intermittent abdominal pressure ventilator for ventilatory support during the procedures.12 The ventilator settings for mouthpiece NIPPV were the same as those for nasal NIPPV. Because supplemental oxygen administration and sedatives can exacerbate hypercapnia and result in ventilatory

FIGURE 4 A 41-yr-old with Duchenne muscular dystrophy dependent on mouthpiece ventilatory support for 27 yrs.

Am. J. Phys. Med. Rehabil. & Vol. 93, No. 2, February 2014

Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.

arrest, end-tidal or transcutaneous carbon dioxide levels may also be monitored along with pulse oximetry with the goal of maintaining normal SpO2 (Q95%) without supplemental oxygen, particularly for patients who are hypercapnic during daytime hours despite ventilator use. Supplemental oxygen was also avoided so that any SpO2 lower than 95% would signal alveolar hypoventilation, and the dental work would be paused so that the patient could eliminate oral air leakage and renormalize SpO2 and alveolar ventilation. Local skin irritation, oropharynx dryness, rhinorrhea, and higher nasal passage air resistance, occasional side effects of prolonged nasal interface use, do not occur with brief use. Patients whose lip strength is inadequate to use mouthpiece NIPPV often use nasal NIPPV around the clock. Such patients can continue nasal ventilation during dental procedures, but if they require general anesthesia, they will often need to be intubated.13,14 They can, however, even with no spontaneous breathing ability, be extubated back to continuous NIPPV after the dental procedures once they meet specific criteria for extubating respirator-dependent patients.15 In conclusion, the nasal interface is generally a viable alternative to intubation or tracheostomy to permit continuous NIPPV during dental procedures for patients who use interfaces that impede oral access.

REFERENCES 1. Bach JR, Martinez D: Duchenne muscular dystrophy: Prolongation of survival by noninvasive interventions. Respir Care 2011;56:744Y50 2. Bach JR, Gupta K, Reyna M, et al: Spinal muscular atrophy type 1: Prolongation of survival by noninvasive respiratory aids. Pediatr Asthma Allergy Immunol 2009;22:151Y62 3. Bach JR: Noninvasive respiratory management and diaphragm and electrophrenic pacing in neuromus-

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cular disease and spinal cord injury. Muscle Nerve 2013;47:297Y305 4. Bach JR, Tilton M: Pulmonary dysfunction and its management in post-polio patients. NeuroRehabilitation 1997;8:139Y53 5. Ishikawa Y, Miura T, Ishikawa Y, et al: Duchenne muscular dystrophy: Survival by cardio-respiratory interventions. Neuromuscul Disord 2011;21:47Y51 6. Bach JR: Home mechanical ventilation for neuromuscular ventilatory failure: Conventional approaches and their outcomes, in Bach JR (ed): Noninvasive Mechanical Ventilation. Philadelphia, PA, Hanley & Belfus, 2002, pp 103Y28 7. Hess DR: The growing role of noninvasive ventilation in patients requiring prolonged mechanical ventilation. Respir Care 2012;57:900Y18 8. Bach JR, Gon0alves MR, Hon AJ, et al: Changing trends in the management of end-stage respiratory muscle failure in neuromuscular disease: Current recommendations of an international consensus. Am J Phys Med Rehabil 2013;92:267Y77 9. Bach JR: A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver. Chest 1993;104:1702Y6 10. Bach JR, Intintola P, Alba AS, et al: The ventilatorassisted individual: Cost analysis of institutionalization versus rehabilitation and in-home management. Chest 1992;101:26Y30 11. Young HK, Lowe A, Fitzgerald DA, et al: Outcome of noninvasive ventilation in children with neuromuscular disease. Neurology 2007;68:198Y201 12. Bach JR, Alba AS: Intermittent abdominal pressure ventilator in a regimen of noninvasive ventilatory support. Chest 1991;99:630Y6 13. Bach JR, Goncalvez M, Sharma A, et al: Open gastrostomy for noninvasive ventilation users with neuromuscular disease. Am J Phys Med Rehabil 2010;89:1Y6 14. Messieha Z: Risks of general anesthesia for the special needs dental patient. Spec Care Dentist 2009;29:21Y5 15. Bach JR, Gon0alves MR, Hamdani I, et al: Extubation of unweanable patients with neuromuscular weakness: A new management paradigm. Chest 2010;137:1033Y9

Mouthpiece NIPPV and Dental Care Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.

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Alternatives to mouthpiece noninvasive ventilatory support to permit dental care.

Patients who are dependent on continuous noninvasive intermittent positive pressure ventilation for ventilatory support via angled mouthpiece interfac...
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