Jared Dyer, DO Profile picture
Oct 31, 2022 15 tweets 9 min read Read on X
1/ Have you ever been confused about the pulmonary management of neuromuscular disorders? If so, I promise you aren’t the only one! Check out this #tweetorial primer on the basics of NMD management. @VCU_PCCM @crit_caring_MD @AvrahamCooperMD @PulmPEEPs #CHESTTrainees #MedEd
2/ Neuromuscular diseases affect the respiratory system in 3 main areas:
1⃣Ventilatory Function
2⃣Cough Function
3⃣Swallowing and Airway Protection

As muscle weakness progresses, this will lead to alveolar hypoventilation with subsequent hypoxemia and hypercapnia.
3/ Objective means to assess respiratory muscle function include:
1⃣Maximal Inspiratory Pressure
2⃣Maximal Expiratory Pressure
3⃣Forced Vital Capacity (Supine)
4⃣Peak Cough Flow

MIP <60 mmHg and nocturnal oximetry are effective in detecting early respiratory insufficiency.
4/ The Canadian Alternatives in Noninvasive Ventilation (CANvent) group developed this staging system for progressive respiratory muscle weakness to help guide clinicians in the management of NMD patients.
5/ Patients will require assisted airway clearance once their peak cough expiratory flow drops below 270 L/min (normal 360-400 L/min). Airway clearance needs to be focused on both the proximal and peripheral airways.
6/ Proximal airway clearance, i.e. cough augmentation, can be achieved through both manual and mechanical means. We will start with the manual methods, which include the following:

1⃣ Manual Breath Stacking
2⃣Resuscitation Bag
3⃣Glossopharyngeal Breathing
7/Glossopharyngeal breathing is a form of positive pressure breathing (i.e. frog breathing) where patients take 25 consecutive small gulps of air (~50mL). This can add up to 1L to the FVC! @atscommunity
atsjournals.org/doi/full/10.11…
8/ Other manual methods for proximal airway clearance include manual cough assist maneuvers:
1⃣Abdominal Thrust
2⃣Chest Wall Thrust
9/ The mechanical insufflator-exsufflator device is the most commonly utilized cough augmentation technique. The insufflator acts as a lung recruitment maneuver while the exsufflator applies negative pressure during exhalation to increase expiratory flow.
10/ Peripheral airway clearance, i.e sputum mobilization, is achieved through the use of high frequency chest wall oscillation. Evidence for vest therapy is conflicting but it should be done in conjunction with cough augmentation.
11/ Worsening respiratory muscle weakness and symptoms of hypoventilation during sleep (daytime somnolence, morning headaches) should prompt a discussion about the use of NIV. Early use of NIV when the FVC <50% and PCO2 > 45mmHg has been shown to improve survival.
12/ This awesome chart by @nickmmark highlights the different forms of non-invasive pressure ventilation. AVAPS (average volume pressure support) is a great starting point for patients with NMD.
13/ AVAPS ensures that patients are receiving a desired tidal volume through dynamic adjustments to the inspiratory pressure (IPAP). This allows for continued delivery of adequate ventilation in the setting of progressive respiratory failure.
14/ Other key points with regards to NIV in neuromuscular disease patients:
1⃣Supplemental oxygen should not be prescribed without ventilatory support as it may mask atelectasis
2⃣Patients need longer inspiratory times to prevent early off-cycling from muscle weakness

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More from @DrJaredDyer

Aug 31, 2021
1/Today’s 🧵 will focus on the pathogenesis of cystic fibrosis along with a brief discussion on the landscape of CFTR modulators. #tweetorial @VCU_PCCM @crit_caring_MD @lkbrath @PSinkam @NaumanChaudary1 @MichaeLheureux @AvrahamCooperMD
2/CF results from a pathogenic mutation on a single gene located on chromosome 7 that encodes the cystic fibrosis transmembrane conductance regulator protein (CFTR). The CFTR serves as a regulated chloride channel that is important in the viscosity of secretions. @mdlizs
3/The CFTR2 database currently reports over 2k different mutations of the CFTR gene; however, the phenotypic expression of these mutations varies based on the functionality of the gene. The most common mutation is the F508del mutation, which is found in ~ 90% of CF patients.
Read 14 tweets
Aug 30, 2021
1/Currently on my cystic fibrosis rotation with @NaumanChaudary1. See below for a brief 🧵from today's learning points on CF diagnosis. This flow diagram from @JPediatr demonstrates the appropriate diagnostic algorithm. #tweetorial @VCU_PCCM @lkbrath @PSinkam @crit_caring_MD
2/Sweat chloride testing can help confirm the diagnosis; however, in individuals with intermediate results (30-59 mmol/L) further testing needs to be completed. These charts from @CF_Foundation highlights sweat chloride testing results.
3/However, sweat chloride testing can result in false positives for a multitude of different reasons. Here a few common causes:
1⃣Adrenal Insufficiency
2⃣Hypothyroidism
3⃣Panhypopituitarism
4⃣Pancreatitis
5⃣Malnutrition/Anorexia
6⃣Glycogen Storage Disorders
7⃣Atopic Dermatitis
Read 4 tweets
Aug 30, 2021
1/Delirium is a common problem that we see in our ICUs. It is associated with the following:

⬆️Mortality
⬆️Length of Stay
⬆️Institutionalization
⬆️Cognitive Decline
⬆️Depression, Anxiety
@VCU_PCCM @lkbrath @crit_caring_MD @PSinkam @VCU_IMRes @mdlizs @MichaeLheureux #MedEd Image
2/A snow globe is a great analogy to discuss and frame delirium with patient’s families. How well the snow is packed at the bottom of the globe can be thought of as predisposing factors. When the snow globe is shaken it represents active delirium from a precipitating cause.
3/Known predisposing factors for delirium include:

1⃣Age >70
2⃣Pre-Existing Dementia
3⃣History of Delirium
4⃣Visual/Hearing Impairment
5⃣Functional Impairment
6⃣Depression
7⃣Polypharmacy
8⃣ETOH/BZD use
@AvrahamCooperMD @aoglasser @ptfaddenMD @michellebr00ks @AdamRodmanMD Image
Read 10 tweets
Oct 16, 2020
1/ Have you ever heard your friendly pulmonologist use the terms “entrapped” or “trapped” lung and were confused about the terminology? If so you are not alone and this is the tweetorial for you! #MedTwitter #tweetorial @crit_caring_MD @lkbrath @VCU_PCCM @PSinkam
2/Let’s first start with some definitions. The term “non-expandable lung” is an umbrella term that is used to describe when the lung is unable to expand to the chest wall in order to achieve visceral and parietal pleura apposition. @AvrahamCooperMD @michellebr00ks @mdlizs
3/There are two pleural diseases that result in the “non-expandable lung”:
1⃣Lung Entrapment
2⃣Trapped Lung
@cjchiu @mkashiouris @AdamRodmanMD @ptfaddenMD @rabihmgeha @DxRxEdu @VCU_IMRes
Read 18 tweets
Sep 25, 2020
1/Pulmonary embolism is a commonly encountered condition; however, how comfortable are you with the acute diagnosis and treatment of the disease? Let's take a walk through the guidelines. #tweetorial #MedEd @VCU_PCCM @crit_caring_MD @lkbrath @PSinkam @AvrahamCooperMD @VCU_IMRes Image
2/ Venous thromboembolism is currently the 3rd most frequent acute cardiovascular syndrome encountered globally with an annual incidence rate ranging from 39-155 per 100,0000 population. bit.ly/3j4iP2s @michellebr00ks @cjchiu @mdlizs @mkashiouris @B_M_Wiese @ptfaddenMD
3/This chart by Rogers, Anderson, and Spencer found in the @EuroRespSoc guidelines displays predisposing risk factors for VTE based on odds ratios. Check out all the risk factors with an odds ratio >10! @DxRxEdu @rabihmgeha @CPSolvers @AdamRodmanMD @tony_breu @KevinSwiatek Image
Read 18 tweets
Aug 28, 2020
1/ What is your differential diagnosis for an individual who is having recurrent asthma exacerbations despite optimal therapy? @VCU_PCCM @crit_caring_MD @PDjack1984 @MichaeLheureux @KevinSwiatek @AndreaMytinger @lkbrath @PSinkam @samirashojaee @AvrahamCooperMD #tweetorial
2/ The differential diagnosis is broad but potential etiologies include:
1⃣ Does this patient actually have asthma?
2⃣ Infectious (bacterial, fungal, viral)
3⃣ GERD
4⃣ Congestive Heart Failure
5⃣ Vocal Cord Dysfunction
@mdlizs @mkashiouris @B_M_Wiese @ptfaddenMD @DxRxEdu
3/ Today we will discuss allergic bronchopulmonary aspergillosis (ABPA), which is an immunological disorder due to hypersensitivity to aspergillus fumigatus. The prevalence of this disease is not widely known; however, literature suggests it be around 13% in asthma clinics.
Read 13 tweets

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