**6. Long term outcomes**

A number of centers have reported up to continuous NIV dependence to maintain patients with neuromuscular disease. One hundred and one of our nocturnal only NIV users became continuously NIV dependent for 7.6 ± 6.1 years to 30.1 ± 6.1 years of age with 56 patients still living. Twenty six became continuously dependent on NIV without requiring hospitalization, while eight continuous tracheostomy ventilation users were decanulated to noninvasive NIV. Using the above described techniques for the DMD patient population, we have extubated 31 "unweanable" intubated patients consecutively to NIV/MAC without resort to tracheotomy (Bach & Martinez, 2001). Also reported by Kohler et al., continuous NIV can prolong life in DMD. Seven of our DMD patients have lived to over 40 years of age requiring continuous NIV for 20 years or more.

Of our 71 SMA type 1 patients sustained with NIV, (mean age 86.1 months (range 13–196) with only 13 deaths at 52.3 months (range 13-111)), fifteen SMA-1 patients are over age 10 and 6 over age 15 without tracheostomy tubes and despite requiring continuous NIV in most cases (Bach et al., 2009). Sixty seven of the patient population could verbally communicate. Schroth also reported use of continuous NIV for SMA type 1 patients (2009). Of seventeen SMA type 1 patients using tracheostomy ventilation with mean age 78.2 months (range 65–179), 25 of 27 lost all autonomous breathing ability immediately upon tracheotomy. Those who had not developed verbalization prior to tracheotomy did not develop vocalization following the procedure. Two brothers with SMA-1 are pictured initially at age 4 then at age 14 and 16 with no VC sustained entirely with NIV (Figure 7 and Figure 8).

instruction of NIV and MAC, and rapid access to MAC during the onset of a chest cold may

Especially in infants and small children, with often inadequate cough to prevent chest colds from triggering pneumonia and ARF, MAC should be used for any desaturation below 95 percent. In continuous NIV users, desaturations are usually due to bronchial mucus plugging, which can develop into atelectasis and pneumonia if the secretions are not quickly cleared.

Certain patient populations are better managed with tracheostomy. Contraindications to noninvasive aid include ventilator dependence with depressed cognitive function, orthopedic conditions interfering with noninvasive interface use, restrictive pulmonary syndrome along with severe pulmonary disease necessitating high FiO2, or uncontrolled seizures or substance abuse (Waldhornet al., 1990). In addition, the presence of a nasogastric tube can hamper the fitting of a nasal interface and the use of mouthpiece or nasal NIV by limiting soft palate closure of the pharynx and the necessary seal at the nose. For neuromuscular disease patients, only those with severe bulbar dysfunction, as observed with severe bulbar ALS resulting in the inability for protect the airway, require tracheotomy (Bach et al., 2004). Other than for the occasional spinal muscular atrophy type 1 patient, tracheotomy is rarely if ever indicated for Duchenne muscular dystrophy or any other neuromuscular disease (Bach et al., 2009). Although tracheostomy ventilation can support alveolar ventilation and extend survival for many NMD patients (Bach, 1996), morbidity and mortality outcomes are not as favorable as by noninvasive approaches (Bach et al., 1998;

A number of centers have reported up to continuous NIV dependence to maintain patients with neuromuscular disease. One hundred and one of our nocturnal only NIV users became continuously NIV dependent for 7.6 ± 6.1 years to 30.1 ± 6.1 years of age with 56 patients still living. Twenty six became continuously dependent on NIV without requiring hospitalization, while eight continuous tracheostomy ventilation users were decanulated to noninvasive NIV. Using the above described techniques for the DMD patient population, we have extubated 31 "unweanable" intubated patients consecutively to NIV/MAC without resort to tracheotomy (Bach & Martinez, 2001). Also reported by Kohler et al., continuous NIV can prolong life in DMD. Seven of our DMD patients have lived to over 40 years of age

Of our 71 SMA type 1 patients sustained with NIV, (mean age 86.1 months (range 13–196) with only 13 deaths at 52.3 months (range 13-111)), fifteen SMA-1 patients are over age 10 and 6 over age 15 without tracheostomy tubes and despite requiring continuous NIV in most cases (Bach et al., 2009). Sixty seven of the patient population could verbally communicate. Schroth also reported use of continuous NIV for SMA type 1 patients (2009). Of seventeen SMA type 1 patients using tracheostomy ventilation with mean age 78.2 months (range 65–179), 25 of 27 lost all autonomous breathing ability immediately upon tracheotomy. Those who had not developed verbalization prior to tracheotomy did not develop vocalization following the procedure. Two brothers with SMA-1 are pictured initially at age 4 then at age 14 and 16 with

be all that is necessary to avert pneumonia, ARF and subsequent hospitalizations.

**5. Contraindications to noninvasive aids: Invasive ventilatory support** 

Toussaint et al., 2006).

**6. Long term outcomes** 

requiring continuous NIV for 20 years or more.

no VC sustained entirely with NIV (Figure 7 and Figure 8).

Fig. 7. Two brothers with spinal muscular atrophy type 1 and continuous ventilator dependence since 4 months of age.

Fig. 8. The same brothers as in Figure 7, now 16 and 14 years of age and with no measurable vital capacity.

In our 176 ALS patients using nocturnal NIV, 42 percent (109 ALS patients) progressed to requiring continuous NIV due to progression of disease, developing severe bulbar innervated muscle impairment that would eventually lead to requiring tracheotomy. Significant aspiration, resulting in consistent baseline SpO2 desaturations to below 95%, due to the weakness of bulbar innervated muscles is the sole indication for tracheotomy in NMD.

Respiratory Muscle Aids in the Management of Neuromuscular

patients to NIV directly to avoid tracheotomy.

**8. "Unweanable" patient decannulation** 

most weaned to only nocturnal NIV.

management and quality of life.

**9. Conclusion** 

them.

dependent.

Respiratory Impairment to Prevent Respiratory Failure and Need for Tracheostomy 249

One hundred and fifty seven consecutive "unweanable" patients were treated including 25 with SMA, 20 with DMD, 16 with ALS, 17 with spinal cord injury, 11 with postpolio syndrome, and 68 with other NMD. Eighty three of these were transferred from other hospitals after refusing tracheostomy after inability to pass spontaneous breathing trials. They were successfully extubated to NIV and MAC despite being unable to pass spontaneous breathing trials before or after extubation. Not requiring re-intubation during the hospitalization defined extubation success. Prior to hospitalization 96 (61%) patients had no experience with NIV, 41 (26%) used it part-time, and 20 (13%) were continuously NIV

There was an extubation success rate of 95% (149 patients) on first attempt. On patients with assisted CPF ≥ 160 L/m, all 98 extubations were successful. Six of 8 patients who had assisted CPF less than 160 L/m initially failed extubation but succeeded on subsequent attempts (Bach et al, 2010). Only two bulbar ALS patients with no measurable assisted CPF underwent tracheotomy (Bach et al., 2010). Multiple centers now routinely extubate DMD

In 1996 we initially reported the decannulation of 50 unweanable patients with neuromuscular weakness (Bach & Saporito, 1996). Any ventilator dependent patient with sufficient bulbar-innervated musculature to prevent significant secretion aspiration is a candidate for decannulation to NIV. This is ideal as decanulation facilitates speech and swallowing. The basic principles for decannulation are essentially identical to those for extubation. Patients with tracheostomy tubes and with no VFBA, possessing VC of 250 mL or greater developed VFBA subsequent to decannulation. Within 3 weeks of decannulation,

The ability to decannulate and maintain survival of neuromuscular disease patients using NIV in place of tracheostomies is indeed possible, and in fact preferable regarding aspects of convenience, speech, swallowing, cosmesis, comfort, safety, and is preferred overall (Bach, 1993). The extubation criteria set forth is a simple evaluation of patients with NMD (ventilatory muscle weakness) compared with the extensive "ventilator weaning parameters" used as criteria along with spontaneous breathing trials considered for the extubation of patients with intrinsic/obstructive lung diseases before extubating

To maintain a sufficient SpO2 of greater than 94 percent, inspiratory and expiratory muscle aid is used to prevent and manage desaturations and hypercapnia instead of supplemental oxygen. "Unweanable" patients, with sufficient glottic function to prevent significant aspiration of secretions, can be extubated to NIV and maintained on NIV for many years, averting tracheostomy. In the NMD patient population, with primarily ventilatory muscle weakness rather than intrinsic lung disease, this alternative paradigm provides optimal
