Diabetic autonomic neuropathy

The British Thoracic Society guideline for respiratory management of children with neuromuscular weakness summarises the available evidence in this field and provides recommendations that will aid healthcare professionals in delivering good quality patient care.

https://thorax.bmj.com/content/thoraxjnl/67/Suppl_1/i1.full.pdf

British Thoracic Society guideline for respiratory management of children with neuromuscular weakness

Chronic respiratory insufficiency is inevitable in the course of disease progression in patients with Duchenne muscular dystrophy (DMD). Without mechanical ventilation (MV), morbidity and mortality are highly likely towards the end of the second decade of life. The present review reports evidence and clinical implications regarding DMD patients treated with MV. There is no doubt that nocturnal hypercapnia precedes daytime hypercapnia. Historical comparisons have provided evidence that non-invasive intermittent positive pressure ventilation (NIPPV) at night is effective and improves quality of life and survival by 5-10 years. By contrast, the optimal criteria and timing for initiation of NIPPV are inconsistent. A recent randomized study however demonstrated the benefits of commencing NIPPV as soon as nocturnal hypoventilation is detected (Ward S, et al., Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax 2005; 60: 1019-24). The respective role of the three hypotheses of the indirect action of nocturnal NIPPV on daytime blood gases may be complimentary; the main improvement may be due to improved ventilatory response to CO2. The ultimate time to offer full time ventilation with the most advantageous interface is lacking in evidence. Full time NIV is possible with a combination of a nasal mask during the night and a mouthpiece during the day, however tracheostomy may be provided when mechanical techniques of cough-assistance are useless to treat chronic cough insufficiency.

https://journals.sagepub.com/doi/pdf/10.1177/1479972307080697

Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: clinical implications of 20 years published experience

Purpose
The purpose of this study is to investigate how respiratory muscle strength correlates to cough capacity in patients with respiratory muscle weakness.

/pdf/how-respiratory-muscle-strength-correlates-with-cough-3xjncksusf.pdf

How respiratory muscle strength correlates with cough capacity in patients with respiratory muscle weakness.

BACKGROUND: Mechanical insufflation/exsufflation (in-exsufflation) increases peak cough flow and may improve sputum clearance. No studies have compared current respiratory physiotherapy practice (manual percussion, shaking, and assisted cough) plus mechanical in-exsufflation to current physiotherapy practice without in-exsufflation in noninvasive-ventilation (NIV) dependent neuromuscular patients with an acute respiratory-tract infection. We hypothesized that airway clearance in these patients would be more effective with in-exsufflation, compared to without in-exsufflation. METHODS: Eight patients (6 male), median age 21.5 y (range 4–44 y). All had sputum retention, with an elevated C-reactive protein of 113 mg/L (range 13–231 mg/L) and a white-cell count of 1410 9 cells/L (range 7–2510 9 cells/L). Patients underwent a 2-day randomized crossover treatment program, with in-exsufflation for one session and without in-exsufflation for the second, with a reverse-crossover on the next day. Treatment time after 30 min (or earlier if sputum clearance was complete) was recorded. Outcome measures were heart rate, pulse oximetry (SpO2 ), transcutaneous carbon dioxide tension (PtcCO2 ), and independently assessed auscultation score. Patients rated effectiveness on a visual analog scale. RESULTS: Treatment time after 30 min was significantly shorter with in-exsufflation, versus without in-exsufflation session (30030 min (range 0–26 min) vs 30 17 47 min (range 0–35 min) (P .03). There was a significant improvement in auscultation score in both groups (with in-exsufflation 2.9 1.9 to 1.8 2.0, P .02; without in-exsufflation 3.4 2.0 to 2.3 2.2, P .007). Visual-analog-scale score for the amount of sputum cleared in both treatment groups showed a decline (P < .05). There was no difference in mean heart rate, SpO2 ,o r PtcCO2 with either treatment. CONCLUSIONS: This is a short-term study of the use of in-exsufflation as a supplement to standard NIV plus physiotherapy in a small group of patients with neuromuscular disease. The addition of mechanical in-exsufflation shortened airway-clearance sessions. The device appeared to be safe and well tolerated, and may provide additional benefit to patients with neuromuscular disease and upper-respiratory-tract

http://rc.rcjournal.com/content/respcare/54/11/1473.full.pdf

The Addition of Mechanical Insufflation/Exsufflation Shortens Airway-Clearance Sessions in Neuromuscular Patients With Chest Infection

The function of inspiratory muscles is crucial for effective cough as well as expiratory muscles in patients with Duchenne muscular dystrophy (DMD). However, there is no report on the correlation between cough and inspiratory muscle strength. To investigate the relationships of voluntary cough capacity, assisted cough techniques, and inspiratory muscle strength as well as expiratory muscle strength in patients with DMD (n= 32). The vital capacity (VC), maximum insufflation capacity (MIC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP) were measured. Unassisted peak cough flow (UPCF) and three different techniques of assisted PCF were evaluated. The mean value of MICs (1918 +/- 586 mL) was higher than that of VCs (1474 +/- 632 mL) (p < 0.001). All three assisted cough methods showed significantly higher value than unassisted method (212 +/- 52 L/min) (F = 66.13, p < 0.001). Combined assisted cough technique (both manual and volume assisted PCF; 286 +/- 41 L/min) significantly exceeded manual assisted PCF (MPCF; 246 +/- 49 L/ min) and volume assisted PCF (VPCF; 252 +/- 45 L/min) (F = 66.13, p < 0.001). MIP (34 +/- 13 cmH2O) correlated significantly with both UPCF and all three assisted PCFs as well as MEP (27 +/- 10 cmH2O) (p < 0.001). Both MEP and MIP, which are the markers of respiratory muscle weakness, should be taken into account in the study of cough effectiveness.

/pdf/respiratory-muscle-strength-and-cough-capacity-in-patients-1bpwbnl8a1.pdf

Respiratory Muscle Strength and Cough Capacity in Patients With Duchenne Muscular Dystrophy

https://www.archives-pmr.org/article/S0003-9993(97)90163-5/pdf

Interlimb interaction and stabilization of contralateral leg in isokinetic knee evaluation

Objective: To investigate the current condition of education and vocation of patients with muscular dystrophy in Korea and to identify the factors determining their educational and vocational status. Method: This study included 129 patients with muscular dystrophy. Functional level of patients was evaluated by modified Barthel index (MBI). The current condition of education and vocation of patients was evaluated by self-reports. The mean age of the patients was 23.5 years and 84.5% were men. Results: Education duration of patients was 11.8 years, similar to that of general Korean population. University graduation rate of patients was 29.8%, which is higher than average rate of general Korean population. The unemployment rate of patients was 77.4%, which is much higher than average rate of general Korean population. Education duration was correlated with the onset age of disease symptom and with the functional level at the end of education period. The vocational status was related with education duration and functional level. The factor which influenced most negatively on maintaining education and vocation was physical limitation. Conclusion: For muscular dystrophy patients with physical limitation, equal opportunities and environment for exercising their ability should be secured with the specific policies and regulations, which consider their physical disabilities and interconnect educational field with vocational field. (J Korean Acad Rehab Med 2008; 32: 51-55)

The Educational and Vocational Status of Muscular Dystrophy Patients

Objective: To evaluate the difference in forced vital capacity (FVC) between sitting and supine position in patients with amyotrophic lateral sclerosis (ALS), cervical spinal cord injury (SCI) and Duchenne muscular dystrophy (DMD). Method: FVC was measured in sitting and supine position for 32 patients with DMD, 32 patients with cervical SCI and for 28 patients with ALS. The highest value in three or more attempts in each position was chosen. Results: FVCs measured in cervical SCI and ALS patients in the sitting and supine position were 1612.8291.0 ml, 1393.2286.7 ml and 2054.7545.8 ml, 1104.3425.4 ml respectively. Cervical SCI patients showed significantly higher value in the supine position (p＜0.05). And ALS patients showed significantly higher value in the sitting position (p＜0.05). FVCs measured in DMD patients were 1311.6260.7 ml and 1213.8378.9 ml respectively. There was no statistically significant difference between the measurements in both positions. Conclusion: Difference in postural change of FVC was observed in patients with different types of neuromuscular disorders. Such difference in FVC suggest that postural change of FVC should be considered in management of neuromuscular disease with respiratory muscle weakness. (J Korean Acad Rehab Med 2006; 30: 80-85)

Change in Forced Vital Capacity with Postures according to Neuromuscular Disease

Objective: To investigate the real condition of pulmonary rehabilitation for patients with advanced neuromuscular diseases (NMDs) on mechanical ventilation in Korea. Method: In order to estimate current state of pulmonary rehabilitative management, chart review and pulmonary function evaluation were conducted in a total of 267 NMD patients who had applied mechanical home ventilator in our center from March 2001 to December 2008. Results: Total 267 patients were included: 95 with Duchenne muscular dystrophy, 69 with other types of myopathy, 83 with amyotrophic lateral sclerosis (ALS), 20 with spinal muscular atrophy. Among them, 18 who were previously intubated and 17 patients who had undergone tracheostomy were switched into volume-limited non-invasive ventilation (NIV). At the time of hospital discharge, 234 patients were applied NIV successfully. Twenty other patients who once used continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) ventilators inappropriately were switched into volume-limited NIV. However, 20 patients who had successfully applied NIV first underwent tracheostomy due to exacerbation of underlying disease. Conclusion: Adequate pulmonary management is the only promising method to prevent lethal complications, and to prolong life span of advanced NMD patients. We assume that more NMD patients can improve their quality of life and prolong their life through proper pulmonary rehabilitation including regular pulmonary function check-ups and ventilatory state monitoring as well as early NIV application.

Jae-Ho Moon

Papers

Respiratory Muscle Strength and Cough Capacity in Patients With Duchenne Muscular Dystrophy

Interlimb interaction and stabilization of contralateral leg in isokinetic knee evaluation

The Educational and Vocational Status of Muscular Dystrophy Patients

Change in Forced Vital Capacity with Postures according to Neuromuscular Disease

A Research on the Management of Ventilatory Insufficiency in Patients with Neuromuscular Diseases