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Paper text:
neuromuscular diseases ( nmd ) , including duchenne muscular dystrophy ( dmd ) , cause 
 ventilatory insufficiency due to weakness of inspiratory muscles , such as the diaphragm1 , 2 . 

moreover , loss 
 of ventilatory muscle strength and laryngopharyngeal function may cause difficulty in 
 expectoration of airway secretions and foreign substances , and acute respiratory failure due 
 to asphyxia or aspiration pneumonia3 . chewing and swallowing problems are common in nmd and reflect the weakness of muscles in 
 the cheeks , lips , tongue and pharynx ; these frequently contribute to malnutrition .
ingestion 
 and swallowing difficulties may occur at a certain age in nmd patients . in spinal muscular 

atrophy , these can occur from infancy to childhood4 , whereas in dmd , swallowing difficulties usually happen after 
 15  years of age , following the appearance of respiratory failure . as a consequence of 
 impaired oropharyngeal function , patients suffer from decreased quality of life ( qol)5 and increased risk for aspiration pneumonia , 
 which might contribute to or exacerbate respiratory failure4 . in recent years ,
noninvasive positive pressure ventilation ( nppv ) has been the first choice 
 for respiratory management of nmd6 .
airway 
 clearance with manually assisted coughing or mechanical insufflation - exsufflation ( mi - e ) had 
 been recommended by guidelines across japan , europe , and united states for patients with 
 impaired cough function to prevent pulmonary complications of asphyxia or aspiration and to 
 avoid tracheostomy or tracheal intubation7,8,9,10,11,12,13 . 
 in fact , these respiratory management strategies have been shown to significantly improve 
 life expectancy and qol of dmd patients14 , 15 .
the aim of this study is to prove that manually assisted coughing or mechanical 
 insufflation - exsufflation prevents pulmonary complication and contribute to continue oral 
 intake safely and continue rate of oral intake in duchenne muscular dystrophy .
the subject was a patient who was able to evaluate respiratory function in dmd patients 
 hospitalized in our hospital at december 2014 .
dmd patients who could not undergo 
 respiratory evaluation due to impaired comprehension , autism , and presence of tracheostomy 
 were excluded .
we reviewed the medical records for variables , such as use of mechanical ventilator , use of 
 mie , and ability for oral intake .
we investigated the duration of four periods of varying 
 cough peak flow ( cpf ) value on unassisted coughing and manually assisted coughing and mi - e 
 use ( fig .
1.different study periods based in cpf value on unassisted coughing and manually 
 assisted coughing and use of mi - e ) .
cpf was measured using a low range peak flow meter ( assess ; healthscan , inc . , cedar 
 grove , nj ) , whereas mi - e was administered by coughassist and coughassist e70 ( philips 
 respironics , inc . ,
this investigation was conducted with the approval of 
 the ethics committee of our hospital ( registration number 28 - 1 ) .
different study periods based in cpf value on unassisted coughing and manually 
 assisted coughing and use of mi - e period a : unassisted cpf 270 l / min before mi - e use .
period b : unassisted cpf<270 l / min and manually assisted cpf 270 l / min before mi - e 
 use .
period c : unassisted cpf<270 l / min and manually assisted cpf<270 l / min with mi - e used 
 for emergency only .
period d : unassisted cpf<270 l / min and manually assisted cpf<270 l / min with constant 
 use of mi - e .
we reviewed the medical records of each period for signs of respiratory tract infection , 
 which was determined based on frequency of fever ( temperature > 37  c ) that required 
 antibiotics .
the variables were compared among the four periods using one - way analysis of 
 variance with bonferroni correction .
statistics analysis was performed using spss ver 22.0  j for windows ( ibm spss 
 japan inc . ,
a total of 58 dmd patients participated in this study ; their average age was 28.7  8.0 
 ( range , 1445 ) years .
ventilatory support was not needed in 6 patients [ age , 18.4  2.7 
 ( range , 1523 ) years ] ; whereas nocturnal nppv was needed in 7 patients [ age , 21.4  5.1 
 ( range , 1428 ) years ] and full - time nppv in 45 patients [ age , 31.2  7.0 ( range , 1745 ) 
 years ] .
all patients who were not on ventilatory support and those on nocturnal nppv were 
 able to tolerate oral intake . among the 45 patients on full - time nppv , 43 ( 95.6% ) were able 
 to continue oral intake for an average of 7.2  4.7  years ( maximum 16.4  years ) , without the 
 need for tracheal intubation due to acute respiratory failure from asphyxia or aspiration 
 pneumonia ( table 1table 1.characteristic of all patients at the end of studyno ventilatory support ( n=6)nocturnal nppv ( n=7)full - time nppv ( n=45)age ( range)18.4  27 ( 1523)21.4  5.1 ( 1428)31.2  7.0 ( 1745)vc ( ml)2,081.7  602.11,118.6  687.3287.4  211.9mic ( ml)2,537.5  179.72,311.4  510.11,703.0  708.7cpf
( l / min)294.2  71.0202.1  96.754.1  62.6assisted cpf ( l / min ) 356.7  51.3324.2  58.6254.2  70.3mi - econstant use0 ( 0%)2 ( 28.6%)35 ( 77.8%)emergency use only6 ( 100%)5 ( 71.4%)10 ( 22.2%)power - enable6 ( 100%)7 ( 100%)42 ( 93.3%)operated vehicledisable0 ( 0%)0 ( 0%)3 ( 6.7%)nutritionoral feeding6 ( 100%)7 ( 100%)43 ( 95.6% ) methodtube feeding0 ( 0%)0 ( 0%)2 ( 4.4%)nppv : noninvasive positive pressure ventilation ; vc : vital capacity ; mic : maximum 
 insufflation capacity ; cpf : cough peak flow ; assisted cpf : manually assisted cough 
 peak flow : mi - e : mechanical insufflation - exsufflation ) . the two remaining full - time nppv patients could not tolerate oral intake and 
 required nasogastric tube feeding due to frequent choking at 33.2 and 36.1  years old , 
 respectively .
nppv : noninvasive positive pressure ventilation ; vc : vital capacity ; mic : maximum 
 insufflation capacity ; cpf : cough peak flow ; assisted cpf : manually assisted cough 
 peak flow : mi - e : mechanical insufflation - exsufflation in all full - time nppv patients , mi - e was introduced when cpf was < 270 l / min . in 35 of 45 

patients , mi - e was used constantly at 35 times / day ( period d ) . in the remaining 10 of 45 
 patients ,
mi - e was unnecessary or was difficult to use continuously every day ; instead , mi - e 
 was used only for emergency situations , such as choking and difficulty in sputum clearance , 
 at a frequency of 01 time / month ( period c ) . in regards to the progression of cpf and mi - e usage , as well as the frequency of fever 
 requiring antibiotics in each period , for periods a , b , c , and d , the cumulative number of 
 patients was 15 , 33 , 12 , and 37 , respectively ; the frequency of fever requiring antibiotics 
 was 39 , 91 , 173 , and 258 , respectively ( table 
 2 
 table 2.frequency of fever needed antibiotics treatment as respiratory tract 
 infections 
 ) .
period c tended to have the highest rate of respiratory infection , but there 
 was no statistically significant difference among the different periods .
in our study , 95.6% of the dmd patients on full - time nppv were able to continue oral 
 nutrition . a large - scale , multicenter study by saitou et al
. showed that despite the use of 
 mechanical ventilation in a majority of hospitalized dmd patients , the rate of oral intake 
 markedly decreased from 95.1% in 1999 to 66.8% in 201216 . on the other hand
, wollinsky reported that only 3 ( 14.3% ) of 21 
 dmd patients using full - time nppv required gastrostomy tube , whereas the remaining 18 
 ( 85.7% ) were able to continue oral nutrition while using nppv17 .
the high rate of oral intake in our study population may be 
 explained by the fact that our patients continued full - time nppv during their meals .
reported that an available mi - e at home was more certain than manually 
 assisted coughing techniques ( i.e. , use of resuscitation bag and chest thrust ) in managing 
 asphyxia due to severe food aspiration in a 29  year - old dmd patient18 . in our hospital , constant use of mi - e or the presence of 
 a staff member who was knowledgeable in mi - e preparation during an emergency may explain the 
 prolonged and safe oral intake of the majority of our patients on full - time nppv .
the frequency of fever requiring antibiotics was the same between periods d and a ( 0.79 vs. 
 0.76 , respectively ) and there was no clear episode of aspiration pneumonia .
bianch compared 
 the data of cpf and other respiratory function tests with and without respiratory 
 complications such as repeated bronchitis and pneumonia at the convalescent stage when 
 restarting oral intake in 55 patients that had cerebrovascular disorder or neurological 
 disorder , with swallowing difficulty confirmed by video fluoroscopic examination ( vf ) .
the 
 results showed that the cpf value of patients with respiratory complications was 
 significantly lower than those without complications , and complications emerged when the 
 value was under 242 l / min19 .
toussaint described that use of daytime nppv may help dmd patients with dysphagia in 
 recovering from dyspnea or respiratory muscle fatigue during a meal .
they recommended 
 respiratory management using nppv and as needed manually 4assisted coughing and noninvasive 
 airway clearance by mi - e to avoid tracheostomy in dmd patients with dysphagia5 . at our hospital , the setting for mi - e was usually at + 40 to + 55 cmh2o positive 
 pressure and 40 to 55 cmh2o negative pressure20 , 21 , and was adjusted to 
 gain enough cpf of 270 l / min ) . in this study , all patients on full - time nppv were noted to 
 have improved cough function from an unassisted cpf of 54.1  62.6 l / min to cpf>270 l / min 
 after use of mi - e .
we considered that the use of mi - e and alteration of food consistency 
 ( pasty or minced ) in these patients were able to prevent pulmonary complications , despite 
 choking or wheezing during a meal .
one limitation of this study was that we were unable to ascertain whether the febrile 
 episodes that required antibiotics were from aspiration pneumonia or from other respiratory 
 tract infections .
the diagnosis of ingestion - swallowing difficulty in patients on full - time 
 nppv was not confirmed by video fluoroscopy ( vf ) .
however , vf is not always useful and an 
 ingestion - swallowing difficulty may be easily observed during a meal and based on changes in 
 ventilation5 , 22 .
use of manually assisted coughing and noninvasive airway clearance by mi - e enabled 
 continuous and safe oral intake in dmd patients on full - time nppv . both nppv and mi - e
are 
 important in avoiding tracheal intubation or tracheostomy and might improve the life 
 expectancy and qol of dmd patients .
[ purpose ] in duchenne muscular dystrophy , it increases risks of difficulties of 
 expectoration of secretion , asphyxia , aspiration pneumonia because of decreased cough 
 function . 
 the aim of this study is to prove that manually assisted coughing or mechanical 
 insufflation - exsufflation prevents pulmonary complication and contribute to continue oral 
 intake safely and continue rate of oral intake in duchenne muscular dystrophy . 
 [ subjects 
 and methods ] we investigated the status of using ventilator , manually assisted coughing or 
 mechanical insufflation - exsufflation , and oral intake or not . in addition , we inspected 
 the frequency of fever ( over 37  c ) needed antibiotics from medical records for index of 
 respiratory tract infection , and compared with every period of using mechanical 
 insufflation - exsufflation from respiratory evaluation on cough peak flow . 
 [ results ] 
 fifty - eight patients participated in this study . 
 there were 45 full - time noninvasive 
 positive pressure ventilation patients . 
 forty - three in 45 full - time noninvasive positive 
 pressure ventilation patients ( 95.6% ) avoided tracheostomy and continued noninvasive 
 positive pressure ventilation because they continued oral intake without tracheal 
 intubation due to the respiratory acute exacerbation by asphyxia or aspiration pneumonia . 
 
 [ conclusion ] duchenne muscular dystrophy patients can continue oral intake safely while 
 preventing pulmonary complication by using manually assisted coughing or mechanical 
 insufflation - exsufflation .