Ventilator Management for Neuromuscular Disease

Hill, Nicholas S.

doi:10.1055/s-2002-33038

Cited by 30 publications

(21 citation statements)

References 24 publications

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“…IPAP is typically increased by 2 -3 cm of H 2 0 as needed to improve symptoms. This is in contrast to some experts who strongly recommend using ''high span'' NIPPV for all ALS patients, defined as greater than 10 cm H 2 0 difference between inspiratory and expiratory pressures, with an average IPAP of 16-18 cm H 2 0 [9,10].…”

Section: Introductioncontrasting

confidence: 57%

Longitudinal assessment of noninvasive positive pressure ventilation adjustments in ALS patients

Gruis

Brown

Lisabeth

et al. 2006

Journal of the Neurological Sciences

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Section: Introductioncontrasting

confidence: 57%

Longitudinal assessment of noninvasive positive pressure ventilation adjustments in ALS patients

Gruis

Brown

Lisabeth

et al. 2006

Journal of the Neurological Sciences

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“…Involvement of the upper airway musculature may produce additional symptoms, such as difficulties with speech or swallowing, and aspiration associated with dysphonia 168. Because muscle weakness contributes to sleep‐disordered breathing, symptoms such as poor sleep, daytime somnolence, and morning headache are common, and often reflective of CO 2 retention 30, 91, 190…”

Section: Detection Of Respiratory Failurementioning

confidence: 99%

Pulmonary complications of chronic neuromuscular diseases and their management

et al. 2003

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Chronic neuromuscular diseases may affect all major respiratory muscles groups including inspiratory, expiratory, and bulbar, and respiratory complications are the major cause of morbidity and mortality. Untreated, many of these diseases lead inexorably to hypercapnic respiratory failure, precipitated in some cases by chronic aspiration and secretion retention or pneumonia, related to impairment of cough and swallowing mechanisms. Many measures are helpful including inhibition of salivation, cough-assist techniques, devices to enhance communication, and physical therapy. In addition, ventilatory assistance is an important part of disease management for patients with advanced neuromuscular disease. Because of its comfort, convenience, and portability advantages, noninvasive positive pressure ventilation (NPPV) has become the modality of first choice for most patients. Patients to receive NPPV should be selected using consensus guidelines, and initiation should be gradual to maximize the chances for success. Attention should be paid to individual preferences for interfaces and early identification of cough impairment that necessitates the use of cough-assist devices. For patients considered unsuitable for noninvasive ventilation, invasive mechanical ventilation should be considered, but only after a frank but compassionate discussion between the patient, family, physician, and other caregivers.

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“…In these cases, prompt respiratory care with pulmonary bi-level positive airway pressure non-invasive ventilation-a common mode of respiratory support for patients with respiratory muscle weakness-can prolong life expectancy. 18 A possible limitation of this study was the fact that histopathological and genetic testing was only performed for a few patients. Further studies are recommended to confirm the histopathological and genetic findings of cases of paediatric RSS in Oman.…”

Section: Discussionmentioning

confidence: 99%

Rigid Spine Syndrome among Children in Oman

Koul

Sankhla

Al-Jahdhami

et al. 2015

Sultan Qaboos Univ Med J

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Objectives: Rigidity of the spine is common in adults but is rarely observed in children. The aim of this study was to report on rigid spine syndrome (RSS) among children in Oman. Methods: Data on children diagnosed with RSS were collected consecutively at presentation between 1996 and 2014 at the Sultan Qaboos University Hospital (SQUH) in Muscat, Oman. A diagnosis of RSS was based on the patient's history, clinical examination, biochemical investigations, electrophysiological findings, neuro-imaging and muscle biopsy. Atrophy of the paraspinal muscles, particularly the erector spinae, was the diagnostic feature; this was noted using magnetic resonance imaging of the spine. Children with disease onset in the paraspinal muscles were labelled as having primary RSS or rigid spinal muscular dystrophy. Secondary RSS was classified as RSS due to the late involvement of other muscle diseases. Results: Over the 18-year period, 12 children were included in the study, with a maleto-female ratio of 9:3. A total of 10 children were found to have primary RSS or rigid spinal muscular dystrophy syndrome while two had secondary RSS. Onset of the disease ranged from birth to 18 months of age. A family history was noted, with two siblings from one family and three siblings from another (n = 5). On examination, children with primary RSS had typical features of severe spine rigidity at onset, with the rest of the neurological examination being normal. Conclusion: RSS is a rare disease with only 12 reported cases found at SQUH during the study period. Cases of primary RSS should be differentiated from the secondary type.

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Ventilator Management for Neuromuscular Disease

Cited by 30 publications

References 24 publications

Longitudinal assessment of noninvasive positive pressure ventilation adjustments in ALS patients

Longitudinal assessment of noninvasive positive pressure ventilation adjustments in ALS patients

Pulmonary complications of chronic neuromuscular diseases and their management

Rigid Spine Syndrome among Children in Oman

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