Non-invasive mechanical ventilation as an alternative respiratory support during gastrostomy tube placement,in a patient with Duchenne muscular dystrophy, 24/24 hours ventilation dependent

Patients with Duchenne muscular dystrophy may benefit from gastrostomy tube feeding due to progressive dysphagia and malnutrition. However, due to concomitant pathologies, they are often at high risk for anesthesiologic complications. We describe how the non-invasive mechanical ventilation has been an alternative successful respiratory support option during the gastrostomy tube placement in a patient with Duchenne muscular dystrophy, on continuous NIV treatment. This report confirms how the use of NIV can support alveolar ventilation, before, during and after mini-invasive procedures, and prevent respiratory complications.Key words: Duchenne muscular dystrophy, percutaneous endoscopic gastrostomy, NIV treatment     

Noninvasive ventilation during gastrostomy tube placement in patients with severe duchenne muscular dystrophy: case reports and review of the literature

Individuals with Duchenne muscular dystrophy may benefit from gastrostomy tube feeding due to progressive dysphagia and malnutrition. However, due to their severely impaired pulmonary function, these individuals are at risk of severe complications when they are sedated or undergo anesthesia for the procedure. We previously described a technique of noninvasive positive pressure ventilation to provide respiratory support during gastrostomy tube placement in such patients, but this technique had risks and limitations. In this case report, we examine two alternative techniques we used to provide respiratory support successfully to patients with severe muscular dystrophy and malnutrition who underwent percutaneous endoscopic gastrostomy tube placement. We then review the literature and discuss the potential benefits, risks, and limitations of the above techniques and of other options for gastrostomy placement in people with severe muscular dystrophy.     

Noninvasive ventilation during percutaneous gastrostomy placement in Duchenne muscular dystrophy

Noninvasive positive pressure ventilation (NPPV) is used for respiratory support in a number of diseases causing acute or chronic respiratory failure. We describe a novel use of NPPV to provide respiratory support during sedation for percutaneous placement of a gastrostomy tube in a patient with Duchenne muscular dystrophy (DMD). The patient had severe respiratory insufficiency, progressive dysphagia, and undernutrition. In addition to the case in this report, we have used NPPV to provide respiratory support to DMD patients during five other gastrointestinal endoscopies without complication. The technique is highly labor intensive and requires physicians and respiratory therapists familiar with NPPV. The primary risk associated with this technique is lack of definitive airway protection during the procedure, which must be balanced against the risks of intubation in an anesthetized patient with neuromuscular disease. The potential benefit to selected patients is substantial, such as initiation of gastrostomy tube feeding in our patient, with subsequent improvement in his quality of life and nutritional status. Pediatr. Pulmonol. 1997; 23:468–471. © 1997 Wiley-Liss, Inc.     

Significance of Medication History at the Time of Entry into the COPDGene Study: Relationship with Exacerbation and CT Metrics

Background: Despite the importance of respiratory medication use in COPD, relatively little is known about which clinical phenotypes were associated with respiratory medications. Methods: To determine the association between respiratory medication use and exacerbations or quantitative CT metrics, we analyzed medication history from 4,484 COPD subjects enrolled in the COPDGene Study. Results: 2,941 (65.6%) subjects were receiving one or more respiratory medications; this group experienced more frequent exacerbations in the year before study entry and had increased gas trapping, emphysema, and subsegmental airway wall area, compared to the patients who were on no respiratory medication. In subgroup analysis, subjects who were on triple therapy (long-acting beta2-agonist [LABA], long-acting muscarinic antagonist [LAMA], and inhaled corticosteroids [ICS]) had the highest frequencies of exacerbations and severe exacerbations and tended to have increased quantitative measures of emphysema and gas trapping on CT compared to other five groups. After adjustment for confounding variables, the triple therapy group experienced more exacerbations and severe exacerbations compared with other five groups. In addition, the LABA+LAMA+ICS group was more likely to have emphysema and gas trapping on CT than other groups in multivariable logistic analysis. Interestingly, the total number of respiratory medications was significantly associated with not only the frequency of exacerbations but also gas trapping and airway wall thickness as assessed by CT scan in multivariable analysis. Conclusions: These results suggest that the use of respiratory medications, especially the number of medications, may identify a more severe phenotype of COPD that is highly susceptible to COPD exacerbations.     

A community-based, time-matched, case-control study of respiratory viruses and exacerbations of COPD

Respiratory viruses are associated with severe acute exacerbations of chronic obstructive pulmonary disease (COPD) in hospitalized patients. However, exacerbations are increasingly managed in the community, where the role of viruses is unclear. In community exacerbations, the causal association between viruses and exacerbation maybe confounded by random fluctuations in the prevalence of circulating respiratory viruses. Therefore, to determine whether viral respiratory tract infections are causally associated with community exacerbations, a time-matched case-control study was performed. Ninety-two subjects (mean age 72 yrs), with moderate to severe COPD, (mean FEV(1) 40% predicted), were enrolled. Nasopharyngeal swabs for viral multiplex polymerase chain reaction and atypical pneumonia serology were obtained at exacerbation onset. Control samples were collected in synchrony, from a randomly selected stable patient drawn from the same cohort. In 99 weeks of surveillance, there were 148 exacerbations. Odds of viral isolation were 11 times higher in cases, than their time-matched controls (34 discordant case-control pairs; in 31 pairs only the case had virus and in three pairs only control). Picornavirus (26), influenza A (3), parainfluenza 1,2,3 (2), respiratory syncytial virus (1), and adenovirus (1) were detected in cases while adenovirus (1) and picornavirus (2) were detected in controls. In patients with moderate or severe COPD the presence of a virus in upper airway secretions is strongly associated with the development of COPD exacerbations. These data support the causative role of viruses in triggering COPD exacerbations in the community.     

Sudden-onset asthma exacerbations: clinical features, response to therapy, and 2-week follow-up. Multicenter Airway Research Collaboration (MARC) investigators.

Sudden-onset asthma exacerbations may have different triggers and responses to treatment than slower-onset exacerbations. The authors studied this hypothesis among patients with severe asthma exacerbations. The Multicenter Airway Research Collaboration prospectively enrolled patients presenting to 64 North American emergency departments with asthma exacerbations. Of 1,847 patients aged 18-54 yrs, 900 had severe exacerbations (peak expiratory flow rate (PEFR) <50% predicted or hospitalized without PEFR). These patients were divided into sudden-onset (< or =3 h of symptoms) and slower-onset (>3 h of symptoms) groups. Fourteen per cent (95% confidence interval, 11-16%) of patients with severe asthma exacerbations had sudden-onset exacerbations. Sudden-onset patients were similar to slower-onset patients, except triggers of their exacerbations were more often respiratory allergens, exercise or psychosocial stress and less often respiratory infections. Sudden-onset patients were more likely to have used oral beta-agonists and salmeterol in the preceding 4 weeks. Although initial PEFRs and management were similar, sudden-onset patients had a greater improvement in PEFR (35 versus 28% p<0.001). Sudden-onset patients were less often discharged on systemic corticosteroids, but had similar 2-week relapse rates compared with slower-onset patients. Among patients presenting with severe asthma exacerbations, sudden-onset exacerbations had a different pattern of triggers and greater improvement with treatment than slower-onset exacerbations.     

The Christmas season as a risk factor for chronic obstructive pulmonary disease exacerbations

BACKGROUND

Epidemics of hospitalization for chronic obstructive pulmonary disease (COPD) occur annually during the Christmas holidays, and COPD exacerbations commonly coincide with respiratory viral infections.

OBJECTIVE

To compare the incidence and determinants of COPD exacerbations occurring between the Christmas holiday period and the remainder of the winter season.

METHODS

Seventy-one subjects with COPD of mixed severity faxed daily symptom diaries to a computer monitoring system from December 1, 2006, to April 30, 2007. Possible exacerbations prompted a home visit for assessment, spirometry and specimen collection for virological testing.

RESULTS

Study subjects submitted a total of 95.4% of possible daily symptom diary sheets by fax. Of 114 possible COPD exacerbations detected using the faxed diaries, 110 met the Anthonisen criteria for true exacerbations. A total of 47 exacerbations (mean 6.7/week) occurred during the Christmas holiday period, while 63 exacerbations (mean 4.3/week) occurred during the remainder of winter. Of the Christmas period exacerbations and of those in the balance of winter, 21 (44%) and 20 (32%), respectively, coincided with respiratory viral infections.

CONCLUSIONS

The incidence of COPD exacerbations during the Christmas period was greater than during the rest of winter in 2006/2007 and peaked immediately before Christmas – in contrast to hospital presentation for COPD, which peaked during the Christmas week. No clear role of respiratory viral infections in the increased rate of exacerbations during the Christmas period was established in the present study. COPD patients were highly compliant with daily symptom reporting using faxed daily diaries, which permitted nearly complete detection of all exacerbations that occurred at incidence.     

Outcomes of Dysphagia Intervention in a Pulmonary Rehabilitation Program

People with chronic obstructive pulmonary disease (COPD) or chronic respiratory disease demonstrate an increased prevalence of oropharyngeal dysphagia as a consequence of impaired coordination between respiration and swallowing function. To date, the effect of patient education and intervention on the management of oropharyngeal dysphagia within pulmonary rehabilitation programs has not been reported or evaluated. Data were collected on participants who were enrolled in the Outpatient Pulmonary Rehabilitation Program and who received dysphagia intervention. Intervention consisted of some or all of the following: (1) a 1-hour dysphagia education program, (2) screening for oropharyngeal dysphagia, and (3) individual comprehensive oropharyngeal dysphagia assessment and management if a screening assessment was failed. A statistically significant improvement was found in participants’ knowledge of dysphagia and COPD (P < 0.001). Participants’ retention of this knowledge 4 days post education remained statistically significant (P < 0.001). Twenty-seven percent of participants who were screened had symptoms of oropharyngeal dysphagia. Fifty-five (53%) participants receiving further individual dysphagia assessment/management correctly completed pre/post swallowing-related quality-of-life surveys (SWAL-QOL). Statistically significant improvement was found in the following subscales: Burden of Dysphagia (P < 0.009), Physical Problems of Dysphagia (P < 0.012) and Managing Diet Options/Food Selection (P < 0.016). Dysphagia education, screening, and management in a pulmonary rehabilitation program improved participants’ swallowing-related quality of life and overall self-management of chronic respiratory disease and dysphagia.     

Evaluation of Respiratory Viral Pathogens in Acute Asthma Exacerbations during Childhood

Objective. Common upper respiratory tract viruses are the most frequent and important causes of asthma exacerbations in both children and adults. Prospective epidemiologic studies report that up to 80% of childhood exacerbations are associated with viral upper respiratory tract infections. Materials and methods. The study group consisted of 104 children with asthma aged 3–17 years who received treatment for asthma exacerbations in our clinic between September 2009 and 2010. Nasopharyngeal and nasal swabs were obtained from all patients during an acute attack, and from the control group (31 subjects). These specimens were investigated for the presence of viral respiratory pathogens using a real-time multiplex PCR method. The patients were compared for the presence of respiratory pathogens and factors related to the severity of the asthma exacerbation. Results. A pathogenic respiratory virus was detected in 53.8% of patients in the acute exacerbation group. The most commonly encountered viral agent was Rhinovirus (35.6%). Patients who had an acute exacerbation with or without a detectable viral pathogen were compared according to the severity of the exacerbation, the need for systemic steroids, and hospitalization rates. No statistically significant difference was found. Conclusion. Although viral upper respiratory tract infections are the most common cause of asthma exacerbations, the severity level of the exacerbation seems to be independent of whether a respiratory virus has been detected.     

Diagnosis and treatment of oculopharyngeal dystrophy: A report of three cases from the same family

Oculopharyngeal muscular dystrophy is a hereditary pathology transmitted in an autosomal dominant manner. The clinical symptoms are palpebral ptosis, oropharyngeal dysphagia and proximal limb weakness. Upper gastro-esophageal endoscopy is recommended to study the dysphagia, a video-radiology study with barium and an esophageal manometry to study the pharyngeo-esophageal motor disorder. Muscle biopsy reveals the presence of atrophic fibers substituted by an increase in fat and connective tissue. In 1998 Brais described the genetic alteration responsible for this pathology, a limited expansion of the triplet of GCG nucleotides in PABP2 gene on chromosome 14q11. Normal individuals have the homozygotic form (GCG)6 of this triplet, whereas patients with the described syndrome have the heterozygotic form (GCG)6-(GCG)9 or (GCG)6-(GCG)10. We present three siblings from the same family with diagnoses and genetic confirmations of oculopharyngeal dystrophy. Two of the patients underwent cricopharyngeal myotomy to relieve the dysphagia.     

Effect of OM-85 BV on reducing bronchiectasis exacerbation in Chinese patients: the iPROBE study

BackgroundBronchiectasis is characterized by recurrent infectious exacerbations. No existing data inform preventive strategy for exacerbations beyond chronic macrolides. OM-85 BV, an immunostimulant, has been shown to prevent recurrent respiratory infections. We initiated this 1-year, multi-centered, double-blind, and controlled trial to investigate the PReventive effect of OM-85 BV on Bronchiectasis Exacerbations in Chinese patients (iPROBE).MethodsPatients with bronchiectasis aged 18 to 75 years, having at least one exacerbation in the past year, were randomized to receive, in addition to any respiratory medications, two courses of 7 mg of OM-85 BV or matching placebo (one capsule orally per day for 10 days a month) for 3 consecutive months, followed by 3 months without treatment. The primary outcomes included the number of acute infectious exacerbations and the time to first exacerbation. Secondary endpoints included patient-reported respiratory outcomes. Safety measures were also assessed.ResultsAmong the 196 participants, 99 were in the OM-85 BV group and 97 in the placebo group. At week 52, the mean number of acute exacerbations per patient was equal to 0.98 and 0.75, respectively, in the two groups (P=0.14). Difference in the time to first pulmonary exacerbation was not statistically significant (P=0.11). There was no statistically significant difference in any secondary end-points. The safety profile in the two arms was good and the majority of adverse events were mild.ConclusionsOM-85 BV did not demonstrate protection in decreasing pulmonary exacerbations of bronchiectasis in this trial performed in Chinese patients. It had good safety profile.     

Oculopharyngeal muscular dystrophy as a cause of dysphagia in the elderly

A case of oculopharyngeal muscular dystrophy in an elderly woman of French-Canadian background presenting with dysphagia is discussed. Typical features of the clinical presentation include bilateral ptosis and dysphagia with significant potential for morbid outcomes of aspiration pneumonia and malnutrition. Although relatively uncommon, this diagnosis should be considered in an elderly person with dysphagia, a history of ptosis, and the proper family background. Surgical treatment appears to improve the signs and symptoms of this disease.     

Meta-analysis: anticholinergics, but not β-agonists, reduce severe exacerbations and respiratory mortality in COPD

BACKGROUND: Anticholinergics and beta2-agonists have generally been considered equivalent choices for bronchodilation in chronic obstructive pulmonary disease (COPD). OBJECTIVE: To assess the safety and efficacy of anticholinergics and beta2-agonists in COPD. DESIGN: We comprehensively searched electronic databases from 1966 to December 2005, clinical trial websites, and references from selected reviews. We included randomized controlled trials of at least 3 months duration that evaluated anticholinergic or beta2-agonist use compared with placebo or each other in patients with COPD. MEASUREMENTS: We evaluated the relative risk (RR) of exacerbations requiring withdrawal from the trial, severe exacerbations requiring hospitalization, and deaths attributed to a lower respiratory event. RESULTS: Pooled results from 22 trials with 15,276 participants found that anticholinergic use significantly reduced severe exacerbations (RR 0.67, confidence interval [CI] 0.53 to 0.86) and respiratory deaths (RR 0.27, CI 0.09 to 0.81) compared with placebo. Beta2-agonist use did not affect severe exacerbations (RR 1.08, CI 0.61 to 1.95) but resulted in a significantly increased rate of respiratory deaths (RR 2.47, CI 1.12 to 5.45) compared with placebo. There was a 2-fold increased risk for severe exacerbations associated with beta2-agonists compared with anticholinergics (RR 1.95, CI 1.39 to 2.93). The addition of beta2-agonist to anticholinergic use did not improve any clinical outcomes. CONCLUSION: Inhaled anticholinergics significantly reduced severe exacerbations and respiratory deaths in patients with COPD, while beta2-agonists were associated with an increased risk for respiratory deaths. This suggests that anticholinergics should be the bronchodilator of choice in patients with COPD, and beta2-agonists may be associated with worsening of disease control.     

Meta-analysis: Anticholinergics, but not β-agonists, reduce severe exacerbations and respiratory mortality in COPD

BACKGROUND: Anticholinergics and β2-agonists have generally been considered equivalent choices for bronchodilation in chronic obstructive pulmonary disease (COPD). OBJECTIVE: To assess the safety and efficacy of anticholinergics and β2-agonists in COPD. DESIGN: We comprehensively searched electronic databases from 1966 to December 2005, clinical trial websites, and references from selected reviews. We included randomized controlled trials of at least 3 months duration that evaluated anticholinergic or β2-agonist use compared with placebo or each other in patients with COPD. MEASUREMENTS: We evaluated the relative risk (RR) of exacerbations requiring withdrawal from the trial, severe exacerbations requiring hospitalization, and deaths attributed to a lower respiratory event. RESULTS: Pooled results from 22 trials with 15,276 participants found that anticholinergic use significantly reduced severe exacerbations (RR 0.67, confidence interval [CI] 0.53 to 0.86) and respiratory deaths (RR 0.27, CI 0.09 to 0.81) compared with placebo. β2-Agonist use did not affect severe exacerbations (RR 1.08, CI 0.61 to 1.95) but resulted in a significantly increased rate of respiratory deaths (RR 2.47, CI 1.12 to 5.45) compared with placebo. There was a 2-fold increased risk for severe exacerbations associated with β2-agonists compared with anticholinergics (RR 1.95, CI 1.39 to 2.93). The addition of β2-agonist to anticholinergic use did not improve any clinical outcomes. CONCLUSION: Inhaled anticholinergics significantly reduced severe exacerbations and respiratory deaths in patients with COPD, while β2-agonists were associated with an increased risk for respiratory deaths. This suggests that anticholinergics should be the bronchodilator of choice in patients with COPD, and β2-agonists may be associated with worsening of disease control.     

The similarities and differences of epidemic cycles of chronic obstructive pulmonary disease and asthma exacerbations

The majority of chronic obstructive pulmonary disease (COPD) and asthma exacerbations in both children and adults are associated with respiratory viral infections and are cyclic in nature. Some variation in these cycles is associated with the timing of the appearance of respiratory viruses, particularly influenza and respiratory syncytial virus. Much more, however, is associated with signal events that are of either fixed or predictable timing. In children, asthma exacerbations reach epidemic levels following school return after the summer vacation and these are predominantly associated with rhinovirus infections. Although younger adults experience a rise in asthma exacerbations at this time, these are secondary to the epidemic in children. Older adults with either COPD or asthma experience only a slightly elevated risk of exacerbations after school return, and hospital presentations for pneumonia in any age group show only marginal increases at that time. Exacerbations of both COPD and adult asthma, with increasing risk with age, are at their highest average annual levels during the Christmas period. This effect appears to be independent of the timing of above average levels of influenza, RSV, parainfluenza, or adenovirus detections; however, hospitalization for respiratory tract infections in all age groups reaches high levels at the same time. Both the post-summer vacation asthma epidemic and the Christmas epidemic of COPD, asthma, and pneumonia are synchronous with the timing of signal events, the day of school return for the former and Christmas Day for the latter, and have been for several years. The agents responsible for the Christmas epidemic of respiratory diseases have not yet been identified. The differences between age and disease exacerbation patterns after school return and at Christmas suggest that either different agents are involved or that the response to a common agent is different between the two signal events.     

Polymyxin B-immobilized fiber columns: A column to breathe new life into the treatment of interstitial lung disease?

Acute exacerbations of idiopathic pulmonary fibrosis (IPF) is a severe respiratory condition with high mortality rate. Direct hemoperfusion with polymyxin B-immobilized fiber columns (PMX-DHP) was originally introduced for the treatment of septic shock. Application of PMX-DHP to the treatment of acute exacerbations of IPF may improve oxygenation and survival of the patients with the disease. In addition to acute exacerbations of IPF, PMX-DHP has been applied to acute respiratory failure from various causes; an amyopathic dermatomyositis patient who developed rapidly progressive interstitial lung disease (ILD) with elevated anti-CADM-140/MDA5 autoantibody and a patient with severe amiodarone pulmonary toxicity. It is also demonstrated that PMX-DHP performed on the first day of steroid pulse therapy may improve the prognosis of patients with rapidly progressive ILDs in a case-control setting. PMX treatment decreases not only various circulating molecules but also inflammatory cells, in particular activated monocytes, producing such mediators. Although the incidence of acute exacerbations of IPF is too low for proper randomization, in order to test the effects of PMX-DHP on the disease, a cohort or case-control analytic study needs to be conducted, preferably from more than one center or research group.     

Early therapy improves outcomes of exacerbations of chronic obstructive pulmonary disease

Treatment of chronic obstructive pulmonary disease (COPD) exacerbations improves outcomes; however, responses to treatment are variable, and patients with COPD often delay presentation or fail to seek therapy. The impact on exacerbation outcomes, hospitalization, and health status of delaying or failing to seek treatment is poorly understood. We studied between 1996 and 2002 a cohort of 128 patients with COPD, mean (SD) FEV(1) of 1.07 (0.43) L. Patients recorded respiratory symptoms daily and reported exacerbations to the outpatient-based study team or to their primary care physician; 1,099 exacerbations were recorded by the patients, of which 658 were reported to a physician. The time between exacerbation onset and treatment was a median (interquartile range) of 3.69 (2.0-5.57) days, and the exacerbation recovery time was 10.7 (7.0-14.0) days. Earlier treatment was associated with a faster recovery (regression coefficient 0.42 days/day delay) (confidence interval, 0.19-0.65; p < 0.001). Patients who reported a higher proportion of exacerbations for treatment had better health-related quality of life than those patients with more untreated exacerbations (rho = -0.22, p = 0.018). Failure to report exacerbations was associated with an increased risk of emergency hospitalization (rho = 0.21, p = 0.04). Patient recognition of exacerbation symptoms and prompt treatment improves exacerbation recovery, reduces risks of hospitalization, and is associated with a better health-related quality of life.     

Long-term follow-up of oropharyngeal dysphagia in children without apparent risk factors

BACKGROUND: The presence of swallowing dysfunction in children without obvious risk factors remains under appreciated. Early identification and prompt initiation of appropriate treatments are critical for reduction of morbidities associated with dysphagia. OBJECTIVE.: To describe the clinical presentations, radiologic characteristics, and long-term outcomes in children with oropharyngeal dysphagia presenting as unexplained respiratory problems. We completed a retrospective chart review of all children without known dysphagic risk factors upon presentation to Speech-Language Pathology (December 1991-April 1995) for feeding/swallowing evaluations because of refractory respiratory problems and dysphagic concerns, and who subsequently were diagnosed with dysphagia on Videofluoroscopic Swallow Study (VFSS). In August 2002, follow-up telephone interviews were conducted with caregivers of 14 children. RESULTS.: We identified 19 children (mean age 1.14 years; range 0.9-5.75) with dysphagia presenting as unexplained respiratory problems. On VFSS, delayed pharyngeal swallow onset was the most common abnormal radiologic finding and always preceded penetration or tracheal aspiration. Eleven (57.9%) children aspirated. Aspiration occurred only with liquids and 100% of aspiration events were silent (i.e., no cough). Dysphagia was not a concern in 11 children at a mean age 3.2 years (range 0.7-10) and persisted in three children who were 9 years or older. CONCLUSIONS.: Oropharyngeal dysphagia should be considered in the differential diagnosis of young children without known risk factors associated with swallowing dysfunction when they present with unexplained respiratory problems. Although the prognosis for resolution of dysphagic concerns is very good, it may take several years.