Use Of BiPAP Research Articles

Long-term myocardial effects of noninvasive ventilation in patients with obesity hypoventilation syndrome

IntroductionChronic effects of noninvasive ventilation on myocardial function in patients with obesity hypoventilation syndrome (OHS) are scarcely understood. The aim of the present study was to evaluate the long-term effects of volume-targeted bilevel positive airway pressure ventilation (BiPAP) on cardiac parameters and myocardial biomarkers in patients with OHS. MethodsClinically stable patients with OHS referred to the tertiary center for the initiation of long-term BiPAP therapy were consecutively enrolled. At baseline, all participants underwent overnight cardiorespiratory polygraphy. BiPAP therapy using volume-targeted spontaneous/timed mode delivered via an oro-nasal mask was initiated. Beat-to-beat noninvasive monitoring by impedance cardiography was used to assess heart function at baseline and after 3 and 12 months of BiPAP use. Serum troponin 1, N-Terminal Pro-B-Type Natriuretic Peptide (NT-ProBNP), tumor necrosis factor-alpha (TNF-α), and interleukin-6 (IL-6) were monitored. ResultsThirteen patients (10 men; mean age, 55.8 ± 9.8 years; mean body mass index of 47.8 ± 5.9 kg/m2) were recruited. From baseline to 3, and to 12 months of BiPAP use, left ventricular stroke volume (SV), ejection time (LVET), and ejection time index significantly increased (P = 0.030; P < 0.001; P = 0.003, respectively), while heart rate and systolic time ratio significantly decreased (P = 0.004; P = 0.034, respectively). Reductions in serum NT-proBNP, IL-6 and TNF-α were observed (P = 0.045; P = 0.018; P = 0.003, respectively). No significant changes in serum troponin were detected throughout the study. ConclusionsThe present findings of increased SV, in association with lengthening of LVET, reductions of NT-proBNP and reductions in circulatory inflammatory markers in patients with stable OHS and chronic moderate-to-severe daytime hypercapnia treated with BiPAP over 1 year support the role of this therapeutic mode in such patients.

Study of 109 Patients Who Required Bipap Therapy During Corona Virus Epidemic

Background :Bipap support is essential for the management of the respiratory failure for the corona virus epidemic. Thestudy focus on the details of the patients, who required bipap support and analyzed characteristics of those patients.Materials and methods: A total of 109 patients were studied for their details including their number of comorbid conditions, age and outcomes. Results: Out of total patients who were discharged after utilizing the bipap support were 58.7% and those who expired were 41.3%. Hence the study shows that the patients who were kept on bipapsupport had shown improvement even after onset of respiratory failure during corona epidemic. The recovery may still depend upon the patients'characteristics including age and number of comorbidities.Conclusion:The patients with higher age and higher number of comorbid conditions like diabetes and hypertension etcmay have higher mortality as compared to the patients having younger age and nil comorbidities. Higher age group patients may carry more chances of poor prognosis. Increased age with higher number of comorbid conditions may lead to higher mortality. However since the ventilated patients have significant high mortality, the bipap therapy still stand a chance to save the patients from being ventilated and may show better outcomes in young patients with less comorbid conditions. The patients presented with worsened respiratory failure at the time of admission and required bipap support immediately, had higher mortality than those who had presented early and then subsequently required further use of bipap after few days of admission. Key words:Respiratory failure, bipap support, age, comorbidities.

SNAP: Implementation of Supportive Non-Invasive Ventilation for Acute Chest Syndrome Prevention in Adults with Sickle Cell Disease

Introduction Acute chest syndrome (ACS) is the 2 nd most common cause of hospitalization and a major cause of mortality in sickle cell disease (SCD). ACS commonly occurs during a hospitalization for a vaso-occlusive crisis (VOC) when the combination of chest wall pain and intravenous opioids may lead to hypoventilation and atelectasis. Incentive spirometry may prevent ACS by encouraging patients to take deep breaths to reduce atelectasis and improve oxygenation, but requires active participation by an awake patient. Non-invasive bi-level positive airway pressure [BiPAP] provides positive pressure breaths via a mask to improve ventilation and prevent atelectasis during sleep. Hospitalized pediatric SCD patients who were at risk for ACS have used nocturnal BiPAP for ACS prevention on the Boston Medical Center (BMC) general pediatric unit since February 2017. In January 2023, BMC instituted the use of nocturnal BiPAP in hospitalized adult patients with SCD who are at high risk for ACS. Methods This is a prospective observational cohort study to examine the safety, acceptability, and tolerability of BiPAP in high-risk adult patients with SCD hospitalized with a VOC. Criteria for increased ACS risk included: 1) Oxygen saturation &amp;lt; 94% on room air at presentation; 2) History of pulmonary hypertension diagnosed by right heart catheterization; 3) Known obstructive sleep apnea (OSA) not currently treated with positive airway pressure; 4) Prior severe ACS requiring invasive or non-invasive ventilation, high-flow oxygen, and/or exchange transfusion; or 5) History of fat emboli syndrome. Exclusion criteria were: 1) OSA being treated with outpatient positive airway pressure, 2) Surgery within the last 30 days, 3) Direct admission to the intensive care unit (ICU). Manual chart extraction was used to collect patient demographic data, medical history, symptoms, vital signs, chest radiography, laboratory data, indications for BiPAP use, and acceptability and use of BiPAP. Results BiPAP was offered during 38 hospitalizations to 25 individual patients (14 (56%) female) between February and July 2023. The mean age was 35.8 years (SD 10.2); 16 (64%) were HbSS, 8 (32%) HbSC and 1 (4%) HbS-β 0. Nineteen patients (76%) were using &amp;gt;1 SCD-modifying therapy; 16 (64%) reported hydroxyurea use. ACS was present on admission in 7 hospitalizations. Patients agreed to try BiPAP in 22/38 hospitalizations (58%) and BiPAP was used for 1 night in 3 hospitalizations, 2 nights in 3 hospitalizations and more than 2 nights in 7 hospitalizations. In 9/22 hospitalizations, attempts at BiPAP use were unsuccessful. Of the 38 hospitalizations in which BiPAP was offered, 3 patients who did not use BiPAP were transferred to the ICU, 2 for ACS and 1 for ketamine infusion for pain that was refractory to high doses of opioids. Three episodes of ACS developed after admission to the hospital: 2 when BiPAP use had not been attempted and 1 when BiPAP was used for 6 days. Blood transfusions were administered during 7 hospitalizations; 5 of which BiPAP was not used. No deaths occurred during this study period. No adverse events were directly attributable to this novel use of BiPAP for ACS prevention. In the 22 encounters where BiPAP use was attempted, four patients noted difficulties with the continuous airflow and feeling suffocated, one reported lightheadedness. One patient who used BiPAP developed oral pain related to periodontal disease. Conclusion Medically stable, adult hospitalized patients with SCD and VOC were willing to try BiPAP to prevent the development of ACS. In 13/22 (59%) BiPAP was tolerated for &amp;gt;1 one night with no adverse events noted. Future directions include optimizing implementation and studying the efficacy of this intervention.

SNAP: Supportive Non-Invasive Ventilation for ACS Prevention in Hospitalized Children with Sickle Cell Disease: Perceptions of Patients, Caregivers, and the Health Care Team

Background: Acute chest syndrome (ACS), the second most common cause of hospitalization and leading cause of death in children with sickle cell disease (SCD), often develops during a hospitalization for acute SCD in the setting of chest wall splinting, hypoventilation, and atelectasis from pain and opioid use. While incentive spirometry is an important component of ACS prevention and management, additional strategies are needed to prevent atelectasis during sleep. Bi-level positive airway pressure ventilation (BiPAP) provides positive pressure breaths through a mask to support ventilation, and is most commonly used to treat respiratory failure and chronic obstructive sleep apnea,. In 2021, we published our clinical innovation using BiPAP during sleep as supportive care during 53 hospitalizations on the general pediatric inpatient unit for hospitalized children with SCD to prevent ACS and/or to prevent acute respiratory failure among children with mild-moderate ACS. We found that our novel use of BiPAP was safe and feasible on a general pediatric unit; when recommended, it was tolerated in 40/53 (75%) hospitalizations. The goal of the current qualitative study was to identify perceived benefits and harms, and facilitators and barriers to the use and widespread implementation of “supportive non-invasive ventilation for ACS prevention” (SNAP) on a general pediatric inpatient unit. Methods: We conducted semi-structured interviews with key implementation partners at Boston Medical Center (pediatric hospitalists and hematologists; nurses, respiratory therapists, child life specialists, clinical leadership, patients, and parents) about their experiences with and perceptions of SNAP. Interviews and a priori codes for the preliminary codebook were guided by the Promoting Action Research on Implementation in Health Services (PARiHS) framework using the Evidence, Context, and Facilitation constructs. Interviews were transcribed, checked for accuracy, and imported into NVivo 12 for analysis. Transcripts were double coded by three team members who independently coded the initial transcripts. The codebook was refined until consensus was reached. Team members independently reviewed data to identify preliminary themes and subsequently consolidated themes as a group. We were particularly interested in themes regarding benefits, advantages, disadvantages, facilitators, and barriers to individual use and institutional implementation of BiPAP as supportive care. Results: Interviews were completed with 16 participants until thematic saturation was reached, including: 5 physicians, 4 nurses, 1 respiratory therapist, 1 child life specialist, 3 parents, and 2 patients. One physician and one nurse had administrative leadership roles. Major themes included: 1) Participants believed that BiPAP is effective at preventing ACS and - when already present - effective at preventing ACS from getting worse. 2) Despite some initial hesitation, inpatient team members endorsed that use of BiPAP on the general pediatric inpatient unit is appropriate. 3) Improving the patient experience with BiPAP is essential and requires a multi-tiered approach including both physical and social components. 4) Communication among inpatient team members and between staff and patients was critical for BiPAP initiation and success. 5) Nurses were key to the intervention success and required additional support. 6) The size of clinical unit and a “buy-in” culture supported intervention success . See Table 1 for detailed subthemes. Conclusions/Future directions: Members of the health care team, patients, and parents perceive SNAP as effective at preventing ACS and respiratory decompensation, and believe that it is appropriate for use with stable hospitalized children at risk for ACS on a general pediatrics inpatient unit. Features of our inpatient pediatrics unit (small size and collaborative relationships between physicians, staff, and nursing leadership) facilitated successful implementation. Improving the patient experience remains a challenge, and will involve technical issues (mask comfort and pressure) and enhanced communication among health care team members and between staff, patients, and caregivers. We are utilizing these data to develop a protocol for a multicenter hybrid effectiveness/implementation trial of SNAP that incorporates these strategies.

1046 Severe hypoventilation in a child with rapid onset weight gain

Abstract Introduction Pediatric sleep-related hypoventilation is a clinical pattern in which ventilatory insufficiency occurs in the setting of either severe OSA (obstructive hypoventilation) or obesity hypoventilation (sleep related hypoventilation). We are reporting a case describing the management of overlapping obstructive hypoventilation due to uncontrolled severe OSA and pediatric obesity hypoventilation syndrome. Report of case(s) The patient is a full term African American 5 y/o male with past medical history of obesity and prediabetes who was referred to discuss sleep related problems after rapid onset of weight gain.He gained 76 lbs over 8-month period; during this time his aunt noticed apneic events with the development of loud snoring.Initial workup was negative for abnormal findings,MRI w/w/o was unremarkable and TSH, cortisol, GH, IGF-1 and prolactin were all within normal limits. X-ray bone age study showed skeletal age appropriate for patient's chronologic age.Upper airway exam showed severe tonsillar hypertrophy 3+ with Mallampati class 4.The diagnostic PSG demonstrated the presence of 12 obstructive, 151 central, and 61 mixed apneas.There were 214 hypopneas resulting in an Apnea\Hypopnea Index of 60.5 events per hour.Mean oxygen saturation was 91.3%.The lowest oxygen saturation during sleep was 64.0%.Time spent ≤88% oxygen saturation was 119.7 minutes (26.2%). End tidal CO2 range was between 50-100 mmHg for 30% of study.The titration study revealed that the optimal treatment modality was BiPAP ST. Conclusion This case focuses on a five-year-old male who experienced rapid onset weight gain and presented with snoring, excessive daytime sleepiness and apneic events and was found to have profound hypoventilation with hypopnea predominant severe obstructive sleep apnea.The initial differential diagnosis of pediatric sleep-related hypoventilation includes ROHHAD, congenital central hypoventilation syndrome, primary central sleep apnea and obesity hypoventilation syndrome.The most common cause of hypoventilation during sleep in children remains OSA.The stepwise approach to management in such a case includes tonsillectomy with adenoidectomy,along with use of BiPAP. After the T&amp;A the patient should have repeat PSG with capnography, if hypercarbia persists on post procedure PSG, he will need to continue BiPAP therapy with appropriate weight loss program. Early recognition evaluation, and treatment of obstructive sleep apnea in the pediatric population is imperative to prevent long-term consequences. Support (if any)

Early initiation of slow continuous ultrafiltration (SCUF) in a case of pregnancy induced hypertension (PIH) with acute decompensated heart failure (ADHF) refractory to standard medical therapy: A case report

Acute pulmonary edema may occur in approximately 3% of women with pre-eclampsia, with 70% of cases occurring after birth. Acute decompensated heart failure (ADHF) patients with refractory edema unresponsive to diuretic therapy may be considered for ultrafiltration. Judicious use of BiPAP with helmet as interface coupled with meticulous hemodynamic monitoring may be a key to successful outcome.

Noninvasive Mechanical Ventilation with Average Volume-Assured Pressure Support versus BiPAP S/T in De Novo Hypoxemic Respiratory Failure.

Background Bilevel positive airway pressure in spontaneous/time and average volume-assured pressure support (BiPAP·S/T–AVAPS) could maintain an adequate tidal volume by reducing the patient's inspiratory effort; however, this ventilatory strategy has not been compared with other ventilatory modes, especially the conventional BiPAP S/T mode, when noninvasive mechanical ventilation (NIMV) is used. The primary objective of this study was to determine the rate of success and failure of the use of BiPAP·S/T-AVAPS versus BiPAP·S/T alone in patients with mild-to-moderate “de novo” hypoxemic respiratory failure. Methods This was a matched-cohort study. Subjects with mild-to-moderate de novo hypoxemic respiratory failure were divided into two groups according to the ventilatory strategy used. The subjects in the BiPAP·S/T group were paired with those in the BiPAP·S/T-AVAPS group. Results A total of 58 subjects were studied. Twenty-nine subjects in the BiPAP·S/T group were paired with 29 subjects in the BiPAP·S/T-AVAPS group. Twenty patients (34.5%) presented with “failure of NIMV,” while 38 (65.5%) patients did not. In addition, 13 (22.4%) patients died, while 45 (77.6%) recovered. No differences were found in the percentage of intubation (P=0.44) and mortality (P=0.1). Conclusion The BiPAP S/T-AVAPS ventilator mode was not superior to the BiPAP·S/T mode. A high mortality rate was observed in patients with NIMV failure in both modes. This trial is registered with https://doi.org/10.1186/ISRCTN17904857.

GIRAFFE IN THE COVID-19 LUNG: PULMONARY FIBROSIS LIMITING SEVERITY OF THE PNEUMOTHORAX

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: More than a year has passed since the first reported cases of COVID-19 worldwide. We present a case of a patient with COVID-19 who acquired bilateral pulmonary fibrosis then later developed a pneumothorax of the right lung. Ironically, it was this fibrosis that prevented a catastrophic collapse of the lung. CASE PRESENTATION: This is a 60-year-old Caucasian female with hypertension, type II diabetes mellitus and obstructive sleep apnea (OSA) who was admitted due to a four-day history of worsening shortness of breath four days due to COVID pneumonia. She was admitted to the ICU due to worsening respiratory status and was maintained on bi-level positive airway pressure ventilation (BIPAP) for two weeks. Repeated chest radiographs in the ICU showed diffuse infiltrates with ground glass opacities bilaterally. She began to improve on the 3rd week of hospitalization, allowing titration down to high flow nasal cannula with BIPAP at night for OSA. On the 4th week, a routine chest radiograph incidentally revealed a small right apical pneumothorax. She had no desaturations, tachypnea, or increased work of breathing at this time. A CT scan of the chest confirmed the extensive pulmonary fibrosis and ground glass opacities and demonstrated the pneumothorax (Figure 1). A repeat 36 hour chest radiograph (Figure 2) showed expansion of the pneumothorax to a volume of 25% of the right hemithorax, with no signs of tension. The patient's clinical status remained unchanged. Follow-up chest CT scan (Figure 1) showed fibrotic attachments tethering the lungs to the pleura that may have limited its further collapse. Patient underwent a CT-guided pigtail catheter insertion the same day. Follow-up imaging the next day confirmed resolution of the pneumothorax. The pigtail catheter was later removed. The patient remained hospitalized, but improving. DISCUSSION: Pulmonary fibrosis is a known sequelae of COVID pneumonia. Bullae and cyst formation can form as a result of this extensive airspace disease. Secondary spontaneous pneumothorax in COVID-19 could be due to damage to the subpleural alveoli from the formation of cysts and bullae leading to spontaneous alveolar rupture into the pleural space. The use of BIPAP could have contributed to it, due to additional volume and barotrauma. Our patient developed pulmonary fibrosis after remaining hospitalized for more than 4 weeks. The fibrosis caused the lung to tether to portions of the pleura, preventing a quick, catastrophic collapse of the lung. The delay in progression gave the patient an opportunity to undergo a CT-guided pigtail catheter insertion, as opposed to an emergent chest tube thoracostomy which would have been a more invasive, traumatic, and painful procedure. CONCLUSIONS: This case highlights the possibility of extensive lung fibrosis, via thick pleural adhesions, protecting patients from developing fatal pneumothorax. REFERENCE #1: Wu, C., et al., Risk Factors Associated With Acute Respiratory Distress Syndrome and Death in Patients With Coronavirus Disease 2019 Pneumonia in Wuhan, China. JAMA Intern Med, 2020. 180(7): p. 934-943. REFERENCE #2: Rai, D.K., P. Sharma, and R. Kumar, Post covid 19 pulmonary fibrosis- Is it reversible? Indian Journal of Tuberculosis, 2020. REFERENCE #3: Martinelli, A.W., et al., COVID-19 and pneumothorax: a multicentre retrospective case series. Eur Respir J, 2020. 56(5) DISCLOSURES: No relevant relationships by Patrick Benjamin, source=Web Response No relevant relationships by Marvyn Allen Chan, source=Web Response No relevant relationships by Josemaria Demigillo, source=Web Response No relevant relationships by Ma Pamela Demigillo, source=Web Response No relevant relationships by Stephen Jesmajian, source=Web Response No relevant relationships by Junfeng Xue, source=Web Response

Stay Current Through Recertification

Stay Current Through Recertification

Non-invasive mechanical ventilation with average volume-assured pressure support. Results according to the aetiology of acute respiratory failure.

Until now, the ventilatory strategy with BiPAP S/T plus average volume-assured pressure support (AVAPS) has not been evaluated for its use in the different types of acute respiratory failure (ARF). Consequently we report the results of the use of this ventilatory strategy in these clinical scenarios. This is asingle-centre prospective study. The subjects were categorised according to the type of ARF: (1) hypercapnic ARF: chronic obstructive pulmonary disease and bronchial asthma; and (2) hypoxaemic ARF: pneumonia, acute respiratory distress syndrome, congestive heart failure, and interstitial lung disease. Multiple logistic regression was used to determine predictors of non-invasive mechanical ventilation (NIV) failure (intubation). Further, in asubgroup of patients with de novo hypoxaemic ARF, analysis of variances with repeated measures was used to determine factors associated with NIV outcome. Sixty-eight subjects were included in this study. The NIV success rate was 69.1% and the mortality rate was 20.6%. Amultivariate analysis showed that the number of affected lung quadrants on chest X-ray (OR: 4.23, 95% CI: 4.17-4.31; P < 0.001) and ARF precipitating disease (OR: 4.46, 95% CI: 4.43-4.51; P < 0.001) were determinants of NIV failure. In the hypoxaemic ARF subgroup (n = 58), significant differences in several parameters were found between patients with positive and negative outcomes. The use of BiPAP S/T - AVAPS in subjects with hypercapnic ARF is associated with abetter outcome than in those with de novo hypoxaemic ARF.

100 Just how long does it take? Using parental surveys documenting time required to provide care at home to children with medical complexity as a tool for advocacy

Abstract Background Children with medical complexity (CMC) constitute a growing population here and everywhere. CMC are fragile, complex, and dependent on technology. Sustainable, safe and effective care at home requires a high level of parental dedication, in knowledge, skills and time. While children thrive in the home setting, the demands on caregivers are substantial. As part of a larger advocacy project, a collaboration between healthcare professionals looking after CMC in Quebec was formed with a primary goal to create evidence-based standardized guidelines for homecare practices. During this process, wide disparities in actual care support received were noted as was the paucity of publication quantifying the time it takes to care for CMC at home. This innovative survey was planned to better describe families’ experiences and to further advocacy efforts with the development of data-driven care support recommendations. Objectives The objectives of this prospective survey were: a) to quantify daily parental expenditure of time to provide care at home to their child requiring i) parenteral nutrition (TPN), ii) peritoneal dialysis (PD), iii) noninvasive ventilation (BIPAP) or iv) tracheostomy care (TC) and b) to qualitatively explore the perceptions of parents regarding existing and desired homecare supports. Design/Methods Quebec CMC were identified through collaboration of multidisciplinary experts from each pediatric university hospital center and the provincial home ventilation program. The daily care required for CMC was described step-by-step with input from CMC’s parents with either TPN, PD, BIPAP or TC. A survey was developed to estimate time required by parents for care completion related to technology, medication, enteral feeding, maintenance of equipment and care organization. Open ended questions on parental perceptions of existing and desired homecare supports were included. The surveys were sent to all eligible families by their primary pediatrician or nurse. Data were entered into a database. For each care intervention, descriptive analyses were performed to determine the median (interquartile range) time required. Thematic analyses were used to evaluate open ended responses. Summary data was used by the expert collaboration to draft data-driven homecare support recommendations for CMC in Quebec and create request template to translate the recommendations into a practical tool for advocacy. Results A total of 87 parents of CMC were surveyed and 78 with complete quantitative data were available for analysis. For TPN, 14 surveys were available. The median (interquartile range) of total daily time dedicated to medical care was 259 (180) minutes a day, with care distributed through TPN administration and preparation (62 (49) minutes/day), enteral nutrition when indicated (42 (34) minutes/day), medication (48 (70) minutes/day) and meal (135(98) minutes/day). For PD, 12 surveys were available. Total daily time dedicated to medical care was 160 (111) minutes/day, with care distributed through dialysis administration and preparation (58 (19) minutes/day), enteral nutrition when indicated (35 (25) minutes/day), medication (35 (28) minutes/day) and other medical care (46 (24) minutes/day). For BIPAP, 39 surveys were available. Total daily time dedicated to care directly associated with BIPAP use was 18 (15) minutes/day. Total daily time dedicated to medical care was 55 (77) minutes/day when well, and 83 (180) minutes/day when sick. Respiratory care was 31 (42) minutes/day when well, and 48 (125) minutes/day when sick. Medication administration was 20 (22) minutes/day when well and 28 (60) minutes/day when sick. Personnel and equipment management was reported to take a median of 55 (interquartile range 119) minutes/week. For TC, 13 surveys were available. Total daily time dedicated to medical care was 109 (239) minutes/day, with 87 (111) minutes/days of direct tracheostomy care. Parents of CMC asked for further support in those categories: financial help, help with home task, respite, nighttime help and specialized support. Conclusion Parents provide substantial care at home for CMC. This study provides a first look at the actual time required to perform care. Although widely distributed, those time data give a first and unique overview of the task for parents taking care of CMC. Data driven recommendations for care support at home were created, along with standardized care request templates which are now in regular use in Quebec, demonstrating a powerful tool for advocacy.

E-POSTERS – EARLY ONSET MUSCLE DISEASE – CASE REPORTS: EP.120ADSSL1 distal myopathy presenting with a more rapid progressive course in patient with trisomy 21

We report a 22 year old female of mixed European and Mexican background with novel compound heterozygous ADSSL1 gene mutations (p.Gly326Asp, pat and c.1202+1G>A, mat) identified by exome sequencing with a more progressive clinical course due to her other diagnosis of trisomy 21 (T21). Her development was appropriate for T21, but she never learned to run, jump or use stairs independently. At age 8 yr, her motor skills started to regress with emergence of weakness. She used a walker by 10 yr and lost ambulation at 12 yr. Currently, she cannot weight-bear (strength 1/5 in most leg muscles; quadriceps at 1-2/5), but has more preserved upper extremity use (strength 3 to 3-/5 range). She has hip, knee contractures, end-grade elbow contractures, and coexistence of finger flexor contractures with laxity in PIP and DIP joints. Her cognitive delays are typical for T21. She developed progressive scoliosis that required T4-L4 spinal fusion at 12 yr of age. There is no cardiomyopathy and her LVSF is 36%. Nocturnal hypoventilation started at age 13 yr with use of BiPAP during sleep since 16 yr of age. Unlike previously reported cases, she had a normal CK and her muscle biopsy at 14 yr did not show rimmed vacuoles or nemaline rods but rather severe myopathic changes: fibrosis and adipose infiltration, variation in fiber size with small, atrophic fibers intermixed with hypertrophic fibers, rounded and splitting fibers, increased central nuclei and occasional degenerating/regenerating fibers. To date, ADSSL1 gene mutations, encoding an enzyme converting inosine monophosphate (IMP) to adenosine monophosphate (AMP), have been reported only in 9 Korean patients from 6 families with 8 of the 9 patients having the same compound heterozygous mutations, suggesting a founder-effect. Identification of another patient with novel mutations suggests that this form of myopathy is panethnic and likely underdiagnosed among patients presenting with signs of generalized and distal myopathy.

Caring Practices May Save a Life.

Caring Practices May Save a Life.

TCTAP C-234 When Catastrophe Happen: Transcatheter Aortic Valve Replacement During Cardiopulmonary Cerebral Resuscitation

### Patient Initials or Identifier Number A.M. Yu ### Relevant Clinical History and Physical Exam A 68-year-old man, a heavy smoker, with a history of; 1. COPD, under BiPAP use at home 2. NSCLC, squamous cell carcinoma, pT2aN1M0, s/p left pneumonectomy, in complete remission He had progressive

Safety and Utility of Bronchoscopy in the Very Elderly

SESSION TITLE: Interventional Pulmonary Procedures SESSION TYPE: Original Investigation Slide PRESENTED ON: Wednesday, November 1, 2017 at 02:45 PM - 04:15 PM PURPOSE: To evaluate if flexible bronchoscopy (FB) in the very elderly provides clinically useful information that affects treatment decisions with an acceptable risk profile. There are several studies evaluating the safety and utility of bronchoscopy in the elderly, but this study describes the oldest population studied to date. METHODS: All bronchoscopies performed in the San Antonio Military Health System over a five year period were reviewed and all patients aged over 85 years old with bronchoscopies performed outside of the ICU were included. A control group of patients aged 65-79 with bronchoscopies performed during the same time period was collected. Procedural data was collected from the electronic medical record including, age at time of procedure, type of bronchoscopy, sampling techniques, medication doses, complications, diagnostic yield, and subsequent treatment. Variables were analyzed with a student’s t-test or a fisher’s exact test. RESULTS: 73 bronchoscopies were performed in the study group, with a mean age of 87.1 (85-96 y). The patient complication rate in the study group was similar to the control group, 9.6% vs 8.2%, p > 0.99. Hypoxia occurred in 5 patients, 3 post procedure ICU transfers, 2 deaths within 30 days, and 1 each of pneumothorax, intubation, bipap use. There were no instances of oversedation requiring reversal agents and no deaths within 72 hours of procedure. Evaluation for malignancy was the most common indication for bronchoscopy(68% vs 59%, p = 0.3) and the most common finding (48% vs 38%, p = 0.24). The study group had statistically significant differences from the control group in the following categories: higher mean age(p < 0.001), higher ASA class(p = 0.03), lower mean midazolam dose (2mg vs 2.6 mg, p = 0.004), and fewer cancer patients who underwent lobectomy (0% vs 5%, p = 0.03). There were no statistically significant differences in the indications for bronchoscopy (p = 0.15), patients undergoing transbronchial biopsy (29% vs 25%, p = 0.7), the diagnostic yield (44% vs 33%, p = 0.17), or the number of patients diagnosed with cancer who received any form of cancer therapy (66% vs 70%, p = 0.79). CONCLUSIONS: This is the oldest cohort of patients undergoing FB studied for procedural complications and diagnostic yield. Despite the fact that the study group was older with a higher average ASA class, the results suggest that bronchoscopy is has essentially the same safety profile for the very elderly. Additionally, it suggests the information obtained from bronchoscopy is utilized in guiding therapy and age does not prevent patients from receiving therapy for malignancy. The lower rate of lobectomies in the study group may reflect poorer functional status and inability to tolerate surgical intervention, or it may be a result of higher TNM stage at time of diagnosis. CLINICAL IMPLICATIONS: These results demonstrate FB in the very elderly has a similar complication rate and diagnostic yield when compared to a younger population, and that malignancy is treated at the same frequency in the very elderly as a younger population. This suggests FB is a safe and efficacious procedure in the very elderly. DISCLOSURE: The following authors have nothing to disclose: Cameron Mclaughlin, Ellis Easterling, Andrew Skabelund No Product/Research Disclosure Information

Application of BiPAP through Endotracheal Tube in Comatose Patients with COPD Exacerbation.

Objective:To evaluate the effectiveness and safety of using BiPAP through endotracheal tube in comatose Chronic Obstructive Pulmonary Disease (COPD) patients with hypercapnic respiratory failure.Methods:This is a prospective study done at Department of Chest Medicine, Jinnah Postgraduate Medical Centre, Karachi, during March to June 2017. It included all comatose COPD patients with hypercapnic respiratory failure who had a poor functional status prior to the illness and who did not meet the criteria to be kept on mechanical ventilator. Patients with apnea and other causes of coma were excluded. These patients were applied BiPAP through endotracheal tube and its response on blood gases and neurological status was evaluated.Results:The success rate of BiPAP through endotracheal tube was 70.5% (31/44). Improvement in Glasgow Coma Scale (GCS) score (p<0.01), pH (p<0.01), and PaCO2 (<0.01) was observed among the responders following two hours and 24 hours of therapy. No significant difference was found in response with regards to gender, smoking status, prior use of noninvasive ventilation or duration of disease. No complications were observed during the therapy.Conclusion:In resource poor settings, the use of BiPAP through endotracheal tube can be an effective and safe intervention for comatose COPD patients with hypercapnic respiratory failure.

Severe Mid-Face Retrognatism following BiPAP use in a Patient with Muscular Dystrophy

Severe Mid-Face Retrognatism following BiPAP use in a Patient with Muscular Dystrophy

Disnea. Insuficiencia respiratoria

Dyspnea is defined as any unpleasant respiratory experience which make a lot of disturbances in the patient's physiology. The perception of dyspnea can differ between individuals, and we might to identify the causes to managing properly. Blood arterial gasometry is crucial to diagnosing respiratory failure, and other parameters such as BNP to guide to cardiac aethiology. Treatment must be applied early providing oxygen through mask or nasal cannula, or in certain settings, through mechanical ventilation. The use of BIPAP is been extending to the emergency departments because of its potential efficacy and lower risks than with invasive ventilation.

Utilização da ventilação não invasiva em edema agudo de pulmão e exacerbação da doença pulmonar obstrutiva crônica na emergência: preditores de insucesso

This study analyzed acute respiratory failure caused by acute pulmonary edema, as well as chronic obstructive pulmonary disease exacerbation, that was treated with non-invasive mechanical ventilation to identify the factors that are associated with the success or failure non-invasive mechanical ventilation in urgent and emergency service. This study was a prospective, descriptive and analytical study. We included patients of both genders aged >18 years who used non-invasive mechanical ventilation due to acute respiratory failure that was secondary to acute pulmonary edema or chronic obstructive pulmonary disease exacerbation. Patients with acute respiratory failure that was secondary to pathologies other than acute pulmonary edema and chronic obstructive pulmonary disease or who presented with contraindications for the technique were excluded. Expiratory pressures between 5 and 8 cmH2O and inspiratory pressures between 10 and 12 cmH2O were used. Supplemental oxygen maintained peripheral oxygen saturation at >90%. The primary outcome was endotracheal intubation. A total of 152 patients were included. The median non-invasive mechanical ventilation time was 6 hours (range 1 - 32 hours) for chronic obstructive pulmonary disease patients (n=60) and 5 hours (range 2 - 32 hours) for acute pulmonary edema patients (n=92). Most (75.7%) patients progressed successfully. However, reduced APACHE II scores and lower peripheral oxygen saturation were observed. These results were statistically significant in patients who progressed to intubation (p<0.001). BiPAP (Bi-level Positive Airway Pressure portable ventilator), as continuous positive airway pressure use increased the probability of endotracheal intubation 2.3 times (p=0.032). Patients with acute pulmonary edema and elevated GCS scores also increased the probability of success. Respiratory frequency >25 rpm, higher APACHE II scores, BiPAP use and chronic obstructive pulmonary disease diagnosis were associated with endotracheal intubation. Higher GCS and SpO2 values were associated with NIV success. Non-invasive mechanical ventilation can be used in emergency services in acute respiratory failure cases caused by acute pulmonary edema and chronic obstructive pulmonary disease exacerbation, but patients with variables related to a higher percentage of endotracheal intubation should be specially monitored.

Effect of Vitamin D Supplementation on ALSFRS in Patients with ALS (P04.152)

Objective: To study the effect of vitamin D (VitD) supplementation on the ALSFRS (Amyotrophic Lateral Sclerosis Functional Rating Scale) in ALS patients. Background ALS patients commonly have low vitD levels. It is not known whether vitD supplementation has an effect on the ALSFRS. Design/Methods: ALSFRS changes in 20 patients with ALS who took 2000IU of vitD daily were compared to 17 patients who didn9t take vitD over 12 months. ALSFRS changes 9 months prior to vitD supplementation were also compared to ensure that the two groups had similar progression prior to starting vitD. Patients were examined every 3 months. Follow-up vitD was obtained 6 months after starting supplementation. Results: ALSFRS decline was slower in patients who took vitD at 3, 6 and 9 months (p=0.04, 0.03 and 0.009 respectively). At 12 months the change in ALSFRS was similar in the 2 groups but follow-up was available for only 6 vitD patients and 7 nonvitD patients (p=0.77). Over 9 months prior to starting vitamin D, the two groups had a similar change in ALSFRS. Other demographics such as age, time since disease onset, vitamin D levels, use of Bipap, Peg tube, FTD, weight loss, ALSFRS at baseline, use of riluzole, depression, smoking and number of patients who died during the study was similar in the 2 groups. Follow-up median vitD levels rose from 18.5 to 31.0 ng/mL at 6 months in the group taking vitD. No side effects secondary to vitD supplementation were reported. Conclusions: VitD supplementation at 2000 IU daily reduced the ALSFRS decline in ALS patients at 3, 6 and 9 months. This effect was lost at 12 months but the number of patients followed at 12 months was small. A larger number of patients is needed to determine whether the beneficial effect of vitD persists after 12 months of supplementation. Disclosure: Dr. Karam has received personal compensation in an editorial capacity for Neurology Resident & Fellow Section. Dr. Barrett has nothing to disclose. Dr. Altememi has nothing to disclose. Dr. Imperato has nothing to disclose. Dr. MacGowan has nothing to disclose. Dr. Scelsa has received personal compensation for activities with Avanir Pharmaceuticals and GlaxoSmithKline. Dr. Scelsa has received personal compensation in an editorial capacity for Peripheral Neuropathies in Clinical Practice. Dr. Scelsa has received research support from the NIH.