**9. Conclusion**

248 Neuromuscular Disorders

Using new NMD specific extubation criteria and protocol, including MAC and pulse oximetry monitoring, "unweanable" patients with DMD, SMA, ALS, and other neuromuscular diseases, e.g., SCI and polio, were successfully extubated to NIV (Bach,

**Extubation Criteria for Unweanable Ventilator Dependent Patients** 

2. PaCO2 40 mm Hg or less at peak inspiratory pressures less than 30 cm H2O on full

4. All oxyhemoglobin desaturations below 95% reversed by mechanically assisted

The extubation criteria and protocol have been developed for the neuromuscular disease specific patient population. Instead of spontaneous breathing trials which patients typically undergo prior to extubation attempts, once a NMD patient meets the criteria cited in Table 1, he or she can be directly extubated to nasal NIV, assist control 800 to 1500 mL and rate 10- 14 breaths/minute in ambient air, with aggressive MAC. Ideally the orogastric or nasogastic tube should be removed to facilitate proper fitting of the NIV interface which can be nasal,

As the patient receives full volume support via NIV, the assisted CPF, or CPF obtained by abdominal thrust following air stacking, is measured within 3 hours of extubation. Patients with sufficient neck movement and lip function used the 15 mm angled mouthpiece and weaned themselves as tolerated by taking fewer and fewer positive pressure ventilations. For those unable to effectively use the 15 mm mouth piece, diurnal nasal NIV was used via nasal prongs, with a nasal or oronasal interface used for nocturnal ventilation. Patients were educated and trained in air stacking and manually assisted coughing and assisted and

For SpO2<95%, ventilator positive inspiratory pressure (PIP), interface or tubing air leakage, CO2 retention, ventilator settings, and MAC were considered. Therapists, nurses, and especially family and personal care attendants were trained and provided with a CoughAssistTM to use MAC via oro-nasal interfaces up to every 30 min until airway secretions cleared and SpO2 could be maintained consistently above 94 percent. Open gastrostomies were performed under local anesthesia using NIV without complication in 7

3. Oxyhemoglobin saturation (SpO2) ≥ 95% for 12 hours or more in ambient air

7. Air leakage via upper airway sufficient for vocalization upon cuff deflation

Table 1. Adapted from Bach, J.R. (2010). Extubation of Patients With Neuromuscular

**7. Extubation of "unweanable" patient** 

1. Afebrile and normal white blood cell count

ventilatory support and normal breathing rate, as needed

5. Fully alert and cooperative, receiving no sedative medications

coughing and suctioning via translaryngeal tube

6. Chest radiograph abnormalities cleared or clearing

Weakness. *Chest, 137( 5), 1033-1039*

oro-nasal and/or mouthpiece interfaces.

unassisted CPF were measured.

patients with unsafe post-extubation oral intake.

2010).

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 them.

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 management and quality of life.

Respiratory Muscle Aids in the Management of Neuromuscular

mobilization. *Chest,* 117:301-303

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Bach, J.R., Rajaraman, R., Ballanger, F., Tzeng, A.C., Ishikawa, Y., Kulessa, R. & Bansal, T.

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Bach, J.R., Smith, W.H., Michaels, J., Saporito, L., Alba, A.S., Dayal, R. & Pan, J. (1993).

Barach, A.L. & Beck, G.J. (1954). Exsufflation with negative pressure: physiologic and

Currie, D.C., Munro, C., Gaskell, D. & Cole PJ. (1986). Practice, problems and compliance

Dail, C.W. & Affeldt, J.E. *Glossopharyngeal breathing [video].* Los Angeles: Department of

Dail, C., Rodgers, M., Guess, V., et al. *Glossopharyngeal breathing.* Downey, CA: Rancho Los

Fishburn, M.J., Marino, R.J., Ditunno, J.F. Jr. (1990). Atelectasis and pneumonia in acute

Garstang, S.V., Kirshblum, S.C. & Wood, K.E. (2000). Patient preference for in-exsufflation for secretion management with spinal cord injury. *J Spinal Cord Med*, 23:80-85 Gomez-Merino, E. & Bach, J.R. (2002). Duchenne muscular dystrophy: prolongation of life by noninvasive respiratory muscle aids. *Am J Phys Med Rehabil,* 81:411-415 Ishikawa, Y. (2005). Manual for the care of patients using noninvasive ventilation. Japan

Kohler, M., Clarenbach, C.F., Böni, L., Brack, T., Russi, E.W., Bloch, K.E. (2005). Quality of

McKim, D.A. & LeBlanc, C. (2006). Maintaining an "oral tradition": specific equipment

life, physical disability, and respiratory impairment in Duchenne muscular

requirements for mouthpiece ventilation instead of tracheostomy for

ventilator assisted individuals. *Arch Phys Med Rehabil*, 74:170-177

bronchiectasis. *Arch Intern Med*, 93:825-841

Visual Education, College of Medical Evangelists, 1954

spinal cord injury. *Arch Phys Med Rehabil*, 71:197-200

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Amigos Department of Physical Therapy, 1979

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**14** 

*Germany* 

**Neuromuscular Diseases in the Context of** 

In this chapter, neuromuscular diseases will be examined from both a psychological and an educational science perspective. Neuromuscular diseases are usually accompanied by many types of psychological strain as functional loss due to immobility or pain often corresponds to emotional impairment, such as fear or depression. The restrictions caused by the disease often remain life-long because as far as current knowledge is concerned no cure has been found yet. Patients' experiences have an immediate impact on both their beliefs about whether and how they can influence the course of their disease, and on their individual perception of their quality of life (Lohaus & Schmitt, 1989). A series of experiments showed that health-related control beliefs and individual quality of life of persons suffering from a serious chronic disease can be lower than of healthy persons (Benassi et al., 1988; Kleftaras, 1997). However, there are hardly any empirical findings pertaining to the area of neuromuscular diseases to this effect. Nevertheless, we may presume that health-related control beliefs and individual quality of life differ between patients with neuromuscular diseases and healthy persons. The following will summarize the findings from two studies, examining the extent of how persons with different neuromuscular diseases differ from healthy persons regarding their evaluation of their individual quality of life and health-

Poverty reports and reports on the correlation between the social situation of people and their health agree that persons with a lower level of education (usually parameterized via the type of graduation achieved) often show a particularly poor state of health, or that they are sicker or die earlier than persons with a higher level of education (Altgeld & Hofrichter, 2000; Jungbauer-Gans & Kriwy, 2003; Richter, 2005; Lambert & Ziese, 2005; Robert Koch Institut & Bundeszentrale für gesundheitliche Aufklärung, 2008). Both health sciences and health politics agree that education by imparting knowledge and promotion of individual disposition and talent support the development of health in childhood and adolescence, and

Almost all epidemiological studies report on social inequality in the sense of unequal access to life opportunities and life risks. Furthermore, data on individual educational biography is

also corresponds to better health in adulthood (Lambert & Ziese, 2005).

**1. Introduction** 

related control beliefs.

**Psychology and Educational Science** 

Andrea Pieter and Michael Fröhlich *Institute for Prevention and Public Health, University of Applied Sciences (DHfPG), Institute for Sport Science, Saarland University* 

