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Replacing the Lung Allocation Score
Diffuse Lung Disease and Lung Transplant Network
Lung Transplant Section
In March 2023, the Composite Allocation Score (CAS) will replace the Lung Allocation Score (LAS) for matching donor lungs to transplant candidates in the United States. The LAS was implemented in 2005 to improve lung organ utilization. Its score was determined by two main factors: (1) risk of 1-year waitlist mortality and (2) likelihood of 1-year post-transplant survival, with the first factor having twice the weight. However, LAS did not account for candidate biology attributes, such as pediatric age, blood type, allosensitization, or height. Long-term survival outcomes under LAS may be reduced, given the greater emphasis on waitlist mortality. Candidates were also subjected to strict geographical distributions within a 250-nautical-mile radius, which frequently resulted in those with lower LAS obtaining a transplant. CAS differs from the LAS in that it assigns an allocation score in a continuous distribution based on the following factors: medical urgency, expected survival benefit following transplant, pediatric age, blood type, HLA antibody sensitization, candidate height, and geographical proximity to the donor organ. Each factor has a specific weight, and because donor factors contribute to CAS, a candidate’s score changes with each donor-recipient match run. Continuous distribution removes hard geographical boundaries and aims for more equitable organ allocation. To understand how allocation might change with CAS, Valapour and colleagues created various CAS scenarios using data from individuals on the national transplant waiting list (Am J Transplant. 2022;22[12]:2971).
They found that waitlist deaths decreased by 36%-47%. This effect was greatest in scenarios where there was less weight on placement efficiency (ie, geography) and more weight on post-transplant outcomes. Transplant system equity also improved in their simulation models. It will be exciting to see how candidate and recipient outcomes are affected once CAS is implemented.
Gloria Li, MD
Member-at-Large
Reference
1. United Network for Organ Sharing. www.unos.org.
Diffuse Lung Disease and Lung Transplant Network
Lung Transplant Section
In March 2023, the Composite Allocation Score (CAS) will replace the Lung Allocation Score (LAS) for matching donor lungs to transplant candidates in the United States. The LAS was implemented in 2005 to improve lung organ utilization. Its score was determined by two main factors: (1) risk of 1-year waitlist mortality and (2) likelihood of 1-year post-transplant survival, with the first factor having twice the weight. However, LAS did not account for candidate biology attributes, such as pediatric age, blood type, allosensitization, or height. Long-term survival outcomes under LAS may be reduced, given the greater emphasis on waitlist mortality. Candidates were also subjected to strict geographical distributions within a 250-nautical-mile radius, which frequently resulted in those with lower LAS obtaining a transplant. CAS differs from the LAS in that it assigns an allocation score in a continuous distribution based on the following factors: medical urgency, expected survival benefit following transplant, pediatric age, blood type, HLA antibody sensitization, candidate height, and geographical proximity to the donor organ. Each factor has a specific weight, and because donor factors contribute to CAS, a candidate’s score changes with each donor-recipient match run. Continuous distribution removes hard geographical boundaries and aims for more equitable organ allocation. To understand how allocation might change with CAS, Valapour and colleagues created various CAS scenarios using data from individuals on the national transplant waiting list (Am J Transplant. 2022;22[12]:2971).
They found that waitlist deaths decreased by 36%-47%. This effect was greatest in scenarios where there was less weight on placement efficiency (ie, geography) and more weight on post-transplant outcomes. Transplant system equity also improved in their simulation models. It will be exciting to see how candidate and recipient outcomes are affected once CAS is implemented.
Gloria Li, MD
Member-at-Large
Reference
1. United Network for Organ Sharing. www.unos.org.
Diffuse Lung Disease and Lung Transplant Network
Lung Transplant Section
In March 2023, the Composite Allocation Score (CAS) will replace the Lung Allocation Score (LAS) for matching donor lungs to transplant candidates in the United States. The LAS was implemented in 2005 to improve lung organ utilization. Its score was determined by two main factors: (1) risk of 1-year waitlist mortality and (2) likelihood of 1-year post-transplant survival, with the first factor having twice the weight. However, LAS did not account for candidate biology attributes, such as pediatric age, blood type, allosensitization, or height. Long-term survival outcomes under LAS may be reduced, given the greater emphasis on waitlist mortality. Candidates were also subjected to strict geographical distributions within a 250-nautical-mile radius, which frequently resulted in those with lower LAS obtaining a transplant. CAS differs from the LAS in that it assigns an allocation score in a continuous distribution based on the following factors: medical urgency, expected survival benefit following transplant, pediatric age, blood type, HLA antibody sensitization, candidate height, and geographical proximity to the donor organ. Each factor has a specific weight, and because donor factors contribute to CAS, a candidate’s score changes with each donor-recipient match run. Continuous distribution removes hard geographical boundaries and aims for more equitable organ allocation. To understand how allocation might change with CAS, Valapour and colleagues created various CAS scenarios using data from individuals on the national transplant waiting list (Am J Transplant. 2022;22[12]:2971).
They found that waitlist deaths decreased by 36%-47%. This effect was greatest in scenarios where there was less weight on placement efficiency (ie, geography) and more weight on post-transplant outcomes. Transplant system equity also improved in their simulation models. It will be exciting to see how candidate and recipient outcomes are affected once CAS is implemented.
Gloria Li, MD
Member-at-Large
Reference
1. United Network for Organ Sharing. www.unos.org.
We need more efforts to prevent sepsis readmissions
Critical Care Network
Sepsis/Shock Section
https://datatools.ahrq.gov/hcup-fast-stats; Kim H, et al. Front Public Health. 2022;10:882715; Torio C, Moore B. 2016. HCUP Statistical Brief #204).
(Since 2013, the Hospital Readmissions Reduction Program (HRRP) adopted pneumonia as a readmission measure, and in 2016, this measure included sepsis patients with pneumonia and aspiration pneumonia. For 2023, the Centers for Medicare and Medicaid Services (CMS) suppressed pneumonia as a readmission measure due to COVID-19’s significant impact (www.cms.gov/Medicare/Medicare-Fee-for-Service-Payment/AcuteInpatientPPS/Readmissions-Reduction-Program). Though sepsis is not a direct readmission measure, it could be one in the future. Studies found higher long-term mortality for patients with sepsis readmitted for recurrent sepsis (Pandolfi F, et al. Crit Care. 2022;26[1]:371; McNamara JF, et al. Int J Infect Dis. 2022;114:34).
A systematic review showed independent risk factors predictive of sepsis readmission: older age, male gender, African American and Asian ethnicities, higher baseline comorbidities, and discharge to a facility. In contrast, sepsis-specific risk factors were extended-spectrum beta-lactamase gram-negative bacterial infections, increased hospital length of stay during initial admission, and increased illness severity (Shankar-Hari M, et al. Intensive Care Med. 2020;46[4]:619; Amrollahi F, et al. J Am Med Inform Assoc. 2022;29[7]:1263; Gadre SK, et al. Chest. 2019;155[3]:483).
McNamara and colleagues found that patients with gram-negative bloodstream infections had higher readmission rates for sepsis during a 4-year follow-up and had a lower 5-year survival rates Int J Infect Dis. 2022;114:34). Hospitals can prevent readmissions by strengthening antimicrobial stewardship programs to ensure appropriate and adequate treatment of initial infections. Other predictive risk factors for readmission are lower socioeconomic status (Shankar-Hari M, et al. Intensive Care Med. 2020;46[4]:619), lack of health insurance, and delays seeking medical care due to lack of transportation (Amrollahi F, et al. J Am Med Inform Assoc. 2022;29[7]:1263).
Sepsis readmissions can be mitigated by predictive analytics, better access to health care, establishing post-discharge clinic follow-ups, transportation arrangements, and telemedicine. More research is needed to evaluate sepsis readmission prevention.
Shu Xian Lee, MD
Fellow-in-Training
Deepa Gotur, MD, FCCP
Member-at-Large
Critical Care Network
Sepsis/Shock Section
https://datatools.ahrq.gov/hcup-fast-stats; Kim H, et al. Front Public Health. 2022;10:882715; Torio C, Moore B. 2016. HCUP Statistical Brief #204).
(Since 2013, the Hospital Readmissions Reduction Program (HRRP) adopted pneumonia as a readmission measure, and in 2016, this measure included sepsis patients with pneumonia and aspiration pneumonia. For 2023, the Centers for Medicare and Medicaid Services (CMS) suppressed pneumonia as a readmission measure due to COVID-19’s significant impact (www.cms.gov/Medicare/Medicare-Fee-for-Service-Payment/AcuteInpatientPPS/Readmissions-Reduction-Program). Though sepsis is not a direct readmission measure, it could be one in the future. Studies found higher long-term mortality for patients with sepsis readmitted for recurrent sepsis (Pandolfi F, et al. Crit Care. 2022;26[1]:371; McNamara JF, et al. Int J Infect Dis. 2022;114:34).
A systematic review showed independent risk factors predictive of sepsis readmission: older age, male gender, African American and Asian ethnicities, higher baseline comorbidities, and discharge to a facility. In contrast, sepsis-specific risk factors were extended-spectrum beta-lactamase gram-negative bacterial infections, increased hospital length of stay during initial admission, and increased illness severity (Shankar-Hari M, et al. Intensive Care Med. 2020;46[4]:619; Amrollahi F, et al. J Am Med Inform Assoc. 2022;29[7]:1263; Gadre SK, et al. Chest. 2019;155[3]:483).
McNamara and colleagues found that patients with gram-negative bloodstream infections had higher readmission rates for sepsis during a 4-year follow-up and had a lower 5-year survival rates Int J Infect Dis. 2022;114:34). Hospitals can prevent readmissions by strengthening antimicrobial stewardship programs to ensure appropriate and adequate treatment of initial infections. Other predictive risk factors for readmission are lower socioeconomic status (Shankar-Hari M, et al. Intensive Care Med. 2020;46[4]:619), lack of health insurance, and delays seeking medical care due to lack of transportation (Amrollahi F, et al. J Am Med Inform Assoc. 2022;29[7]:1263).
Sepsis readmissions can be mitigated by predictive analytics, better access to health care, establishing post-discharge clinic follow-ups, transportation arrangements, and telemedicine. More research is needed to evaluate sepsis readmission prevention.
Shu Xian Lee, MD
Fellow-in-Training
Deepa Gotur, MD, FCCP
Member-at-Large
Critical Care Network
Sepsis/Shock Section
https://datatools.ahrq.gov/hcup-fast-stats; Kim H, et al. Front Public Health. 2022;10:882715; Torio C, Moore B. 2016. HCUP Statistical Brief #204).
(Since 2013, the Hospital Readmissions Reduction Program (HRRP) adopted pneumonia as a readmission measure, and in 2016, this measure included sepsis patients with pneumonia and aspiration pneumonia. For 2023, the Centers for Medicare and Medicaid Services (CMS) suppressed pneumonia as a readmission measure due to COVID-19’s significant impact (www.cms.gov/Medicare/Medicare-Fee-for-Service-Payment/AcuteInpatientPPS/Readmissions-Reduction-Program). Though sepsis is not a direct readmission measure, it could be one in the future. Studies found higher long-term mortality for patients with sepsis readmitted for recurrent sepsis (Pandolfi F, et al. Crit Care. 2022;26[1]:371; McNamara JF, et al. Int J Infect Dis. 2022;114:34).
A systematic review showed independent risk factors predictive of sepsis readmission: older age, male gender, African American and Asian ethnicities, higher baseline comorbidities, and discharge to a facility. In contrast, sepsis-specific risk factors were extended-spectrum beta-lactamase gram-negative bacterial infections, increased hospital length of stay during initial admission, and increased illness severity (Shankar-Hari M, et al. Intensive Care Med. 2020;46[4]:619; Amrollahi F, et al. J Am Med Inform Assoc. 2022;29[7]:1263; Gadre SK, et al. Chest. 2019;155[3]:483).
McNamara and colleagues found that patients with gram-negative bloodstream infections had higher readmission rates for sepsis during a 4-year follow-up and had a lower 5-year survival rates Int J Infect Dis. 2022;114:34). Hospitals can prevent readmissions by strengthening antimicrobial stewardship programs to ensure appropriate and adequate treatment of initial infections. Other predictive risk factors for readmission are lower socioeconomic status (Shankar-Hari M, et al. Intensive Care Med. 2020;46[4]:619), lack of health insurance, and delays seeking medical care due to lack of transportation (Amrollahi F, et al. J Am Med Inform Assoc. 2022;29[7]:1263).
Sepsis readmissions can be mitigated by predictive analytics, better access to health care, establishing post-discharge clinic follow-ups, transportation arrangements, and telemedicine. More research is needed to evaluate sepsis readmission prevention.
Shu Xian Lee, MD
Fellow-in-Training
Deepa Gotur, MD, FCCP
Member-at-Large
Closer to home: Melioidosis in the United States
Chest Infections & Disaster Response Network
Disaster Response & Global Health Section
caused by the gram-negative bacillus Burkholderia pseudomallei, does not usually appear on the differential diagnosis of patients in the United States. Historically endemic to South and Southeast Asia, Australia, Puerto Rico, and Central America, B. pseudomallei infects humans via direct inoculation of the skin, through inhalation, or by the ingestion of contaminated soil or water. Importation of melioidosis to the United States from civilian travelers, global commerce, or military personnel is becoming more common (Gee JE, et al. N Engl J Med.A case series of four patients across four states occurred in 2021. Contaminated aromatherapy sprays sold from a retailer whose supplier originated from India were identified as the source (Gee JE, et al). Two additional cases were reported in Mississippi spanning 2 years (CDC Health Alert Network. July 27, 2022). A case in Texas describes the zoonotic detection of the organism in a raccoon carcass (Petras JK, et al. MMWR. 2022;71:1597). Now, cases of U.S. domestic melioidosis have been described, with the CDC identifying areas of the Mississippi Gulf Coast as an endemic region.
The gold standard of diagnosis is the isolation of B. pseudomallei in culture. Serologic tests may also be useful. Automated bacterial identification systems may provide initially inaccurate results, delaying diagnosis and increasing mortality. Presenting symptoms are nonspecific and may resemble typical sepsis syndromes, as well as cavitary lung disease, mimicking TB. The diagnosis requires a high index of suspicion with targeted interviewing.
Clinicians should reevaluate patients with isolates identified as Burkholderia species, especially those who are unresponsive to standard empiric therapies. Treatment for melioidosis involves initial antibiotic therapy with ceftazidime, meropenem, or imipenem, followed by eradication therapy with trimethoprim-sulfamethoxazole or amoxicillin-clavulanate for up to 6 months (Wiersinga WJ, et al. N Engl J Med. 2012;367[11]:1035).
Zein Kattih, MD
Chest Infections & Disaster Response Network
Disaster Response & Global Health Section
caused by the gram-negative bacillus Burkholderia pseudomallei, does not usually appear on the differential diagnosis of patients in the United States. Historically endemic to South and Southeast Asia, Australia, Puerto Rico, and Central America, B. pseudomallei infects humans via direct inoculation of the skin, through inhalation, or by the ingestion of contaminated soil or water. Importation of melioidosis to the United States from civilian travelers, global commerce, or military personnel is becoming more common (Gee JE, et al. N Engl J Med.A case series of four patients across four states occurred in 2021. Contaminated aromatherapy sprays sold from a retailer whose supplier originated from India were identified as the source (Gee JE, et al). Two additional cases were reported in Mississippi spanning 2 years (CDC Health Alert Network. July 27, 2022). A case in Texas describes the zoonotic detection of the organism in a raccoon carcass (Petras JK, et al. MMWR. 2022;71:1597). Now, cases of U.S. domestic melioidosis have been described, with the CDC identifying areas of the Mississippi Gulf Coast as an endemic region.
The gold standard of diagnosis is the isolation of B. pseudomallei in culture. Serologic tests may also be useful. Automated bacterial identification systems may provide initially inaccurate results, delaying diagnosis and increasing mortality. Presenting symptoms are nonspecific and may resemble typical sepsis syndromes, as well as cavitary lung disease, mimicking TB. The diagnosis requires a high index of suspicion with targeted interviewing.
Clinicians should reevaluate patients with isolates identified as Burkholderia species, especially those who are unresponsive to standard empiric therapies. Treatment for melioidosis involves initial antibiotic therapy with ceftazidime, meropenem, or imipenem, followed by eradication therapy with trimethoprim-sulfamethoxazole or amoxicillin-clavulanate for up to 6 months (Wiersinga WJ, et al. N Engl J Med. 2012;367[11]:1035).
Zein Kattih, MD
Chest Infections & Disaster Response Network
Disaster Response & Global Health Section
caused by the gram-negative bacillus Burkholderia pseudomallei, does not usually appear on the differential diagnosis of patients in the United States. Historically endemic to South and Southeast Asia, Australia, Puerto Rico, and Central America, B. pseudomallei infects humans via direct inoculation of the skin, through inhalation, or by the ingestion of contaminated soil or water. Importation of melioidosis to the United States from civilian travelers, global commerce, or military personnel is becoming more common (Gee JE, et al. N Engl J Med.A case series of four patients across four states occurred in 2021. Contaminated aromatherapy sprays sold from a retailer whose supplier originated from India were identified as the source (Gee JE, et al). Two additional cases were reported in Mississippi spanning 2 years (CDC Health Alert Network. July 27, 2022). A case in Texas describes the zoonotic detection of the organism in a raccoon carcass (Petras JK, et al. MMWR. 2022;71:1597). Now, cases of U.S. domestic melioidosis have been described, with the CDC identifying areas of the Mississippi Gulf Coast as an endemic region.
The gold standard of diagnosis is the isolation of B. pseudomallei in culture. Serologic tests may also be useful. Automated bacterial identification systems may provide initially inaccurate results, delaying diagnosis and increasing mortality. Presenting symptoms are nonspecific and may resemble typical sepsis syndromes, as well as cavitary lung disease, mimicking TB. The diagnosis requires a high index of suspicion with targeted interviewing.
Clinicians should reevaluate patients with isolates identified as Burkholderia species, especially those who are unresponsive to standard empiric therapies. Treatment for melioidosis involves initial antibiotic therapy with ceftazidime, meropenem, or imipenem, followed by eradication therapy with trimethoprim-sulfamethoxazole or amoxicillin-clavulanate for up to 6 months (Wiersinga WJ, et al. N Engl J Med. 2012;367[11]:1035).
Zein Kattih, MD
Use of low-cost air quality monitors for patients with lung disease
DIFFUSE LUNG DISEASE & LUNG TRANSPLANT NETWORK
Occupational & Environmental Health Section
The World Health Organization estimates significant air pollution–attributable deaths, including 11% of lung cancer deaths, 18% of COPD deaths, and 23% of pneumonia deaths (www.who.org). (Carlsten C, et al. Europ Respir J. 2020;55[6]: 1902056).
The Environmental Protection Agency uses air quality (AQ) monitors around the country to track ambient pollution levels. These real-time data are available to the public on AirNow.gov; however, these data do not reflect indoor air pollutants. Thus, AQ monitors may not accurately represent the total air pollution exposure to patients.
Low-cost AQ monitors available for purchase enable indoor AQ monitoring.
Unfortunately, many indoor air pollutants do not have well-established safe levels. Although several devices detect specific pollutants like volatile oxygen compounds or particulate matter, other harmful compounds may remain undetectable and unmonitored. Even if high pollutant levels are detected, most devices are not designed to alarm like smoke and carbon monoxide detectors (www.epa.gov).
Although efficacy data are limited, several laboratories, such as the Indoor Environment Lab at Berkeley, have conducted performance evaluations. In a study of 16 devices publicly available for purchase, the devices tended to underreport pollutant levels by nearly 50%. Nevertheless, most devices successfully detected the presence of pollutants (Demanega I, et al. Building and Environment. 2021;187:107415).
Regardless of these limitations, low-cost AQ monitors may empower patients to intervene on unsafe household conditions and minimize their risk of poor lung health.
Alexys Monoson, MD
Section Fellow-in-Training
DIFFUSE LUNG DISEASE & LUNG TRANSPLANT NETWORK
Occupational & Environmental Health Section
The World Health Organization estimates significant air pollution–attributable deaths, including 11% of lung cancer deaths, 18% of COPD deaths, and 23% of pneumonia deaths (www.who.org). (Carlsten C, et al. Europ Respir J. 2020;55[6]: 1902056).
The Environmental Protection Agency uses air quality (AQ) monitors around the country to track ambient pollution levels. These real-time data are available to the public on AirNow.gov; however, these data do not reflect indoor air pollutants. Thus, AQ monitors may not accurately represent the total air pollution exposure to patients.
Low-cost AQ monitors available for purchase enable indoor AQ monitoring.
Unfortunately, many indoor air pollutants do not have well-established safe levels. Although several devices detect specific pollutants like volatile oxygen compounds or particulate matter, other harmful compounds may remain undetectable and unmonitored. Even if high pollutant levels are detected, most devices are not designed to alarm like smoke and carbon monoxide detectors (www.epa.gov).
Although efficacy data are limited, several laboratories, such as the Indoor Environment Lab at Berkeley, have conducted performance evaluations. In a study of 16 devices publicly available for purchase, the devices tended to underreport pollutant levels by nearly 50%. Nevertheless, most devices successfully detected the presence of pollutants (Demanega I, et al. Building and Environment. 2021;187:107415).
Regardless of these limitations, low-cost AQ monitors may empower patients to intervene on unsafe household conditions and minimize their risk of poor lung health.
Alexys Monoson, MD
Section Fellow-in-Training
DIFFUSE LUNG DISEASE & LUNG TRANSPLANT NETWORK
Occupational & Environmental Health Section
The World Health Organization estimates significant air pollution–attributable deaths, including 11% of lung cancer deaths, 18% of COPD deaths, and 23% of pneumonia deaths (www.who.org). (Carlsten C, et al. Europ Respir J. 2020;55[6]: 1902056).
The Environmental Protection Agency uses air quality (AQ) monitors around the country to track ambient pollution levels. These real-time data are available to the public on AirNow.gov; however, these data do not reflect indoor air pollutants. Thus, AQ monitors may not accurately represent the total air pollution exposure to patients.
Low-cost AQ monitors available for purchase enable indoor AQ monitoring.
Unfortunately, many indoor air pollutants do not have well-established safe levels. Although several devices detect specific pollutants like volatile oxygen compounds or particulate matter, other harmful compounds may remain undetectable and unmonitored. Even if high pollutant levels are detected, most devices are not designed to alarm like smoke and carbon monoxide detectors (www.epa.gov).
Although efficacy data are limited, several laboratories, such as the Indoor Environment Lab at Berkeley, have conducted performance evaluations. In a study of 16 devices publicly available for purchase, the devices tended to underreport pollutant levels by nearly 50%. Nevertheless, most devices successfully detected the presence of pollutants (Demanega I, et al. Building and Environment. 2021;187:107415).
Regardless of these limitations, low-cost AQ monitors may empower patients to intervene on unsafe household conditions and minimize their risk of poor lung health.
Alexys Monoson, MD
Section Fellow-in-Training
2023 GOLD update: Changes in COPD nomenclature and initial therapy
AIRWAYS DISORDERS NETWORK
Asthma & COPD Section
The 2023 GOLD committee proposed changes in nomenclature and therapy for various subgroups of patients with COPD. The 2023 GOLD committee changed the ABCD group classification to ABE (for exacerbations), which highlights the importance of the number and severity of exacerbations irrespective of daily symptoms.
People with “young COPD” develop respiratory symptoms and meet spirometric criteria for COPD between the ages of 25 and 50 years old. Other terminology changes center around those with functional and/or structural changes suggesting COPD, but who do not meet the post-bronchodilator spirometric criteria to confirm the COPD diagnosis.
Those with “pre-COPD” have normal spirometry, including the FEV1 and FEV1/FVC ratio, but have functional and/or structural changes concerning for COPD. Functional changes include air trapping and/or hyperinflation on PFTs, low diffusion capacity, and/or decline in FEV1 of >40 mL per year.
Structural changes include emphysematous changes and/or bronchial wall changes on CT scans. “PRISm” stands for preserved ratio with impaired spirometry, where the postbronchodilator FEV1/FVC is greater than or equal to 0.70, but FEV1 is < 80% predicted with similar functional and/or structural changes to those with “pre-COPD.” People with PRISm have increased all-cause mortality. Not all people with pre-COPD or PRISm progress clinically and spiro-metrically to COPD; however, they should be treated because they have symptoms as well as functional and/or structural abnormalities. Despite increasing data regarding pre-COPD and PRISm, many gaps remain regarding optimal management.
Maria Ashar, MD, MBBS
Section Fellow-in-Training
REFERENCE
Global strategy for prevention, diagnosis and management of COPD: 2023 report; https://goldcopd.org. Accessed March 13, 2023.
AIRWAYS DISORDERS NETWORK
Asthma & COPD Section
The 2023 GOLD committee proposed changes in nomenclature and therapy for various subgroups of patients with COPD. The 2023 GOLD committee changed the ABCD group classification to ABE (for exacerbations), which highlights the importance of the number and severity of exacerbations irrespective of daily symptoms.
People with “young COPD” develop respiratory symptoms and meet spirometric criteria for COPD between the ages of 25 and 50 years old. Other terminology changes center around those with functional and/or structural changes suggesting COPD, but who do not meet the post-bronchodilator spirometric criteria to confirm the COPD diagnosis.
Those with “pre-COPD” have normal spirometry, including the FEV1 and FEV1/FVC ratio, but have functional and/or structural changes concerning for COPD. Functional changes include air trapping and/or hyperinflation on PFTs, low diffusion capacity, and/or decline in FEV1 of >40 mL per year.
Structural changes include emphysematous changes and/or bronchial wall changes on CT scans. “PRISm” stands for preserved ratio with impaired spirometry, where the postbronchodilator FEV1/FVC is greater than or equal to 0.70, but FEV1 is < 80% predicted with similar functional and/or structural changes to those with “pre-COPD.” People with PRISm have increased all-cause mortality. Not all people with pre-COPD or PRISm progress clinically and spiro-metrically to COPD; however, they should be treated because they have symptoms as well as functional and/or structural abnormalities. Despite increasing data regarding pre-COPD and PRISm, many gaps remain regarding optimal management.
Maria Ashar, MD, MBBS
Section Fellow-in-Training
REFERENCE
Global strategy for prevention, diagnosis and management of COPD: 2023 report; https://goldcopd.org. Accessed March 13, 2023.
AIRWAYS DISORDERS NETWORK
Asthma & COPD Section
The 2023 GOLD committee proposed changes in nomenclature and therapy for various subgroups of patients with COPD. The 2023 GOLD committee changed the ABCD group classification to ABE (for exacerbations), which highlights the importance of the number and severity of exacerbations irrespective of daily symptoms.
People with “young COPD” develop respiratory symptoms and meet spirometric criteria for COPD between the ages of 25 and 50 years old. Other terminology changes center around those with functional and/or structural changes suggesting COPD, but who do not meet the post-bronchodilator spirometric criteria to confirm the COPD diagnosis.
Those with “pre-COPD” have normal spirometry, including the FEV1 and FEV1/FVC ratio, but have functional and/or structural changes concerning for COPD. Functional changes include air trapping and/or hyperinflation on PFTs, low diffusion capacity, and/or decline in FEV1 of >40 mL per year.
Structural changes include emphysematous changes and/or bronchial wall changes on CT scans. “PRISm” stands for preserved ratio with impaired spirometry, where the postbronchodilator FEV1/FVC is greater than or equal to 0.70, but FEV1 is < 80% predicted with similar functional and/or structural changes to those with “pre-COPD.” People with PRISm have increased all-cause mortality. Not all people with pre-COPD or PRISm progress clinically and spiro-metrically to COPD; however, they should be treated because they have symptoms as well as functional and/or structural abnormalities. Despite increasing data regarding pre-COPD and PRISm, many gaps remain regarding optimal management.
Maria Ashar, MD, MBBS
Section Fellow-in-Training
REFERENCE
Global strategy for prevention, diagnosis and management of COPD: 2023 report; https://goldcopd.org. Accessed March 13, 2023.
Training the future cardiac intensivist to meet the demands of the modern cardiovascular ICU
PULMONARY VASCULAR & CARDIOVASCULAR NETWORK
Cardiovascular Medicine & Surgery Section
Over the recent decades, the cardiovascular intensive care unit (CICU) has seen a significant transformation. Not only has the acuity of cardiac conditions evolved, but the prevalence of noncardiac critical illness has multiplied (Yuriditsky E, et al. ATS Sch. 2022;3[4]):522).
2012;126:1408).
However, fewer than 15% of modern CICUs are staffed by physicians dual-boarded in cardiology and CCM; most believe that CCM training is necessary to effectively practice in the CICU (van Diepen S, et al. Circ Cardiovasc Qual Outcomes. 2017;10:e003864).
How best do we develop future cardiac intensivists to manage complex decompensated cardiovascular disease with compounded medical critical illness?
Multiple training pathways leading to board eligibility and dual certification have been outlined (Geller BJ, et al. J Am Coll Cardiol. 2018;72:1171). A commonly elected path requires the completion of a 1-year CCM fellowship following a 3-year general cardiology fellowship.
As few programs exist, limited guidance is available surrounding CCM fellowship design for the cardiologist; however, proposed curricula have been published (Yuriditsky E, et al. ATS Sch. 2022;3[4]:522).
Developing such programs requires collaboration between cardiologists and intensivists to secure funding, develop infrastructure, obtain accreditation, and to recruit candidates.
Having completed dual training, I not only saw my skillset flourish, but the partnership between CCM and cardiology strengthen. As interest in this field grows, we eagerly await to see program adaptation and innovative curriculum design.
Eugene Yuriditsky, MD
Section Fellow-in-Training
PULMONARY VASCULAR & CARDIOVASCULAR NETWORK
Cardiovascular Medicine & Surgery Section
Over the recent decades, the cardiovascular intensive care unit (CICU) has seen a significant transformation. Not only has the acuity of cardiac conditions evolved, but the prevalence of noncardiac critical illness has multiplied (Yuriditsky E, et al. ATS Sch. 2022;3[4]):522).
2012;126:1408).
However, fewer than 15% of modern CICUs are staffed by physicians dual-boarded in cardiology and CCM; most believe that CCM training is necessary to effectively practice in the CICU (van Diepen S, et al. Circ Cardiovasc Qual Outcomes. 2017;10:e003864).
How best do we develop future cardiac intensivists to manage complex decompensated cardiovascular disease with compounded medical critical illness?
Multiple training pathways leading to board eligibility and dual certification have been outlined (Geller BJ, et al. J Am Coll Cardiol. 2018;72:1171). A commonly elected path requires the completion of a 1-year CCM fellowship following a 3-year general cardiology fellowship.
As few programs exist, limited guidance is available surrounding CCM fellowship design for the cardiologist; however, proposed curricula have been published (Yuriditsky E, et al. ATS Sch. 2022;3[4]:522).
Developing such programs requires collaboration between cardiologists and intensivists to secure funding, develop infrastructure, obtain accreditation, and to recruit candidates.
Having completed dual training, I not only saw my skillset flourish, but the partnership between CCM and cardiology strengthen. As interest in this field grows, we eagerly await to see program adaptation and innovative curriculum design.
Eugene Yuriditsky, MD
Section Fellow-in-Training
PULMONARY VASCULAR & CARDIOVASCULAR NETWORK
Cardiovascular Medicine & Surgery Section
Over the recent decades, the cardiovascular intensive care unit (CICU) has seen a significant transformation. Not only has the acuity of cardiac conditions evolved, but the prevalence of noncardiac critical illness has multiplied (Yuriditsky E, et al. ATS Sch. 2022;3[4]):522).
2012;126:1408).
However, fewer than 15% of modern CICUs are staffed by physicians dual-boarded in cardiology and CCM; most believe that CCM training is necessary to effectively practice in the CICU (van Diepen S, et al. Circ Cardiovasc Qual Outcomes. 2017;10:e003864).
How best do we develop future cardiac intensivists to manage complex decompensated cardiovascular disease with compounded medical critical illness?
Multiple training pathways leading to board eligibility and dual certification have been outlined (Geller BJ, et al. J Am Coll Cardiol. 2018;72:1171). A commonly elected path requires the completion of a 1-year CCM fellowship following a 3-year general cardiology fellowship.
As few programs exist, limited guidance is available surrounding CCM fellowship design for the cardiologist; however, proposed curricula have been published (Yuriditsky E, et al. ATS Sch. 2022;3[4]:522).
Developing such programs requires collaboration between cardiologists and intensivists to secure funding, develop infrastructure, obtain accreditation, and to recruit candidates.
Having completed dual training, I not only saw my skillset flourish, but the partnership between CCM and cardiology strengthen. As interest in this field grows, we eagerly await to see program adaptation and innovative curriculum design.
Eugene Yuriditsky, MD
Section Fellow-in-Training
2023 GOLD update: Changes in COPD nomenclature and initial therapy
Airways Disorders Network
Asthma & COPD Section
The 2023 GOLD committee proposed changes in nomenclature and therapy for various subgroups of patients with COPD.
The mainstay of initial treatment for symptomatic COPD should include combination LABA/LAMA bronchodilators in a single inhaler. For patients with features of concomitant asthma or eosinophils greater than or equal to 300 cells/microliter, an ICS/LABA/LAMA combination inhaler is recommended.
People with “young COPD” develop respiratory symptoms and meet spirometric criteria for COPD between the ages of 25 and 50 years old. Other terminology changes center around those with functional and/or structural changes suggesting COPD, but who do not meet the postbronchodilator spirometric criteria to confirm the COPD diagnosis.
Those with “pre-COPD” have normal spirometry, including the FEV1 and FEV1/FVC ratio, but have functional and/or structural changes concerning for COPD. Functional changes include air trapping and/or hyperinflation on PFTs, low diffusion capacity, and/or decline in FEV1 of > 40 mL per year.
Structural changes include emphysematous changes and/or bronchial wall changes on CT scans. “PRISm” stands for preserved ratio with impaired spirometry, where the postbronchodilator FEV1/FVC is greater than or equal to 0.70, but FEV1 is < 80% predicted with similar functional and/or structural changes to those with “pre-COPD.” People with PRISm have increased all-cause mortality. Not all people with pre-COPD or PRISm progress clinically and spirometrically to COPD; however, they should be treated because they have symptoms as well as functional and/or structural abnormalities. Despite increasing data regarding pre-COPD and PRISm, many gaps remain regarding optimal management.
Maria Ashar, MD, MBBS
Airways Disorders Network
Asthma & COPD Section
The 2023 GOLD committee proposed changes in nomenclature and therapy for various subgroups of patients with COPD.
The mainstay of initial treatment for symptomatic COPD should include combination LABA/LAMA bronchodilators in a single inhaler. For patients with features of concomitant asthma or eosinophils greater than or equal to 300 cells/microliter, an ICS/LABA/LAMA combination inhaler is recommended.
People with “young COPD” develop respiratory symptoms and meet spirometric criteria for COPD between the ages of 25 and 50 years old. Other terminology changes center around those with functional and/or structural changes suggesting COPD, but who do not meet the postbronchodilator spirometric criteria to confirm the COPD diagnosis.
Those with “pre-COPD” have normal spirometry, including the FEV1 and FEV1/FVC ratio, but have functional and/or structural changes concerning for COPD. Functional changes include air trapping and/or hyperinflation on PFTs, low diffusion capacity, and/or decline in FEV1 of > 40 mL per year.
Structural changes include emphysematous changes and/or bronchial wall changes on CT scans. “PRISm” stands for preserved ratio with impaired spirometry, where the postbronchodilator FEV1/FVC is greater than or equal to 0.70, but FEV1 is < 80% predicted with similar functional and/or structural changes to those with “pre-COPD.” People with PRISm have increased all-cause mortality. Not all people with pre-COPD or PRISm progress clinically and spirometrically to COPD; however, they should be treated because they have symptoms as well as functional and/or structural abnormalities. Despite increasing data regarding pre-COPD and PRISm, many gaps remain regarding optimal management.
Maria Ashar, MD, MBBS
Airways Disorders Network
Asthma & COPD Section
The 2023 GOLD committee proposed changes in nomenclature and therapy for various subgroups of patients with COPD.
The mainstay of initial treatment for symptomatic COPD should include combination LABA/LAMA bronchodilators in a single inhaler. For patients with features of concomitant asthma or eosinophils greater than or equal to 300 cells/microliter, an ICS/LABA/LAMA combination inhaler is recommended.
People with “young COPD” develop respiratory symptoms and meet spirometric criteria for COPD between the ages of 25 and 50 years old. Other terminology changes center around those with functional and/or structural changes suggesting COPD, but who do not meet the postbronchodilator spirometric criteria to confirm the COPD diagnosis.
Those with “pre-COPD” have normal spirometry, including the FEV1 and FEV1/FVC ratio, but have functional and/or structural changes concerning for COPD. Functional changes include air trapping and/or hyperinflation on PFTs, low diffusion capacity, and/or decline in FEV1 of > 40 mL per year.
Structural changes include emphysematous changes and/or bronchial wall changes on CT scans. “PRISm” stands for preserved ratio with impaired spirometry, where the postbronchodilator FEV1/FVC is greater than or equal to 0.70, but FEV1 is < 80% predicted with similar functional and/or structural changes to those with “pre-COPD.” People with PRISm have increased all-cause mortality. Not all people with pre-COPD or PRISm progress clinically and spirometrically to COPD; however, they should be treated because they have symptoms as well as functional and/or structural abnormalities. Despite increasing data regarding pre-COPD and PRISm, many gaps remain regarding optimal management.
Maria Ashar, MD, MBBS
Breathing easier: The growing adoption of indwelling pleural catheters
Thoracic Oncology Network
Interventional Procedures Section
The management of recurrent pleural effusions is challenging. 2018;198[7]:839) and when talc pleurodesis is unsuccessful in patients with an expandable lung (Dresler CM, et al. Chest. 2005;127[3]:909).
These devices have become an important treatment option in patients with malignant pleural effusions (MPE), particularly those with a nonexpandable lung (Feller-Kopman DJ, et al. Am J Respir Crit Care Med.Over the last 5 years, studies evaluating the use of IPCs in treating nonmalignant pleural disease have proliferated. These studies have included and shown the successful treatment of pleural effusions due to end-stage renal disease, advanced heart failure (Walker SP, et al. Eur Respir J. 2022;59[2]:2101362), and cirrhosis, especially when a transjugular intrahepatic portosystemic shunt or liver transplant is not an option (Shojaee S, et al., Chest. 2019;155[3]:546). Compared with MPE, the rate of pleurodesis is generally lower and takes longer when an IPC is used to manage a nonmalignant pleural disease. Infection is the most common complication; most cases can be managed without catheter removal.
With many cited advantages, the IPC is an essential tool in the armamentarium of the chest physician and interventional radiologist. Indwelling pleural catheters have proven applications beyond MPE. When applied in a multidisciplinary fashion involving subspecialists and considering the patient’s goals, using an IPC can help achieve a crucial patient-centric goal in managing a recurrent nonmalignant pleural effusion.
Samiksha Gupta, MD
2nd Year Fellow
Sameer Kaushik Avasarala, MD
Section Member-at-Large
Thoracic Oncology Network
Interventional Procedures Section
The management of recurrent pleural effusions is challenging. 2018;198[7]:839) and when talc pleurodesis is unsuccessful in patients with an expandable lung (Dresler CM, et al. Chest. 2005;127[3]:909).
These devices have become an important treatment option in patients with malignant pleural effusions (MPE), particularly those with a nonexpandable lung (Feller-Kopman DJ, et al. Am J Respir Crit Care Med.Over the last 5 years, studies evaluating the use of IPCs in treating nonmalignant pleural disease have proliferated. These studies have included and shown the successful treatment of pleural effusions due to end-stage renal disease, advanced heart failure (Walker SP, et al. Eur Respir J. 2022;59[2]:2101362), and cirrhosis, especially when a transjugular intrahepatic portosystemic shunt or liver transplant is not an option (Shojaee S, et al., Chest. 2019;155[3]:546). Compared with MPE, the rate of pleurodesis is generally lower and takes longer when an IPC is used to manage a nonmalignant pleural disease. Infection is the most common complication; most cases can be managed without catheter removal.
With many cited advantages, the IPC is an essential tool in the armamentarium of the chest physician and interventional radiologist. Indwelling pleural catheters have proven applications beyond MPE. When applied in a multidisciplinary fashion involving subspecialists and considering the patient’s goals, using an IPC can help achieve a crucial patient-centric goal in managing a recurrent nonmalignant pleural effusion.
Samiksha Gupta, MD
2nd Year Fellow
Sameer Kaushik Avasarala, MD
Section Member-at-Large
Thoracic Oncology Network
Interventional Procedures Section
The management of recurrent pleural effusions is challenging. 2018;198[7]:839) and when talc pleurodesis is unsuccessful in patients with an expandable lung (Dresler CM, et al. Chest. 2005;127[3]:909).
These devices have become an important treatment option in patients with malignant pleural effusions (MPE), particularly those with a nonexpandable lung (Feller-Kopman DJ, et al. Am J Respir Crit Care Med.Over the last 5 years, studies evaluating the use of IPCs in treating nonmalignant pleural disease have proliferated. These studies have included and shown the successful treatment of pleural effusions due to end-stage renal disease, advanced heart failure (Walker SP, et al. Eur Respir J. 2022;59[2]:2101362), and cirrhosis, especially when a transjugular intrahepatic portosystemic shunt or liver transplant is not an option (Shojaee S, et al., Chest. 2019;155[3]:546). Compared with MPE, the rate of pleurodesis is generally lower and takes longer when an IPC is used to manage a nonmalignant pleural disease. Infection is the most common complication; most cases can be managed without catheter removal.
With many cited advantages, the IPC is an essential tool in the armamentarium of the chest physician and interventional radiologist. Indwelling pleural catheters have proven applications beyond MPE. When applied in a multidisciplinary fashion involving subspecialists and considering the patient’s goals, using an IPC can help achieve a crucial patient-centric goal in managing a recurrent nonmalignant pleural effusion.
Samiksha Gupta, MD
2nd Year Fellow
Sameer Kaushik Avasarala, MD
Section Member-at-Large
Early mobility in the ICU: Working with the TEAM
Critical Care Network
Nonrespiratory Critical Care Section
2014;370:1626). This advocacy is endorsed by major societies and guidelines, like the ABCDEF bundle (Balas MC, et al. Crit Care Med. 2013;41:S116), in which “E” stands for Early mobility and exercise. In fact, the PADIS guidelines, addressing Pain, Agitation, Delirium, Immobility, and Sleep in the ICU, added Immobility and Sleep (the “I” and “S” in PADIS) to the prior PAD guidelines in the latest update in 2018, to stress the importance of early mobility in the ICU (Devlin JW, et al. Crit Care Med. 2018;46[9]:e825). Multiple studies have shown a positive impact of early mobility in the ICU on patients’ outcomes (Tipping CJ, et al. Intensive Care Med. 2017;43:171).
This is especially true for critically ill patients, in which weakness is more common and can result in worse outcomes (Kress JP, et al. N Engl J Med.The recent TEAM study examined an early mobility approach in mechanically ventilated patients and found no difference in the primary outcome of alive and out-of-hospital at 180 days (N Engl J Med. 2022;387:1747).
Before concluding, it is worth realizing that the usual care arm included mobilization that was otherwise normally provided. The intervention arm protocolized the early mobility to be done simultaneously with the minimization of sedation. Patients’ assessment occurred in 81% in the usual care arm vs 94% in the intervention arm; both numbers are much higher than reported data in the ICU (Jolley SE, et al. Crit Care Med. 2017;45:205).
Revisiting the question of early mobility in the ICU, more data are needed to clarify the best methodology, sedation, timing, amount, and type of patients who will benefit the most. Until then, it should remain a goal for ICUs and part of the daily discussion when caring for critically ill patients.
Mohammed J. Al-Jaghbeer, MBBS, FCCP
Section Member-at-Large
Salim Surani, MD, MPH, FCCP
Critical Care Network
Nonrespiratory Critical Care Section
2014;370:1626). This advocacy is endorsed by major societies and guidelines, like the ABCDEF bundle (Balas MC, et al. Crit Care Med. 2013;41:S116), in which “E” stands for Early mobility and exercise. In fact, the PADIS guidelines, addressing Pain, Agitation, Delirium, Immobility, and Sleep in the ICU, added Immobility and Sleep (the “I” and “S” in PADIS) to the prior PAD guidelines in the latest update in 2018, to stress the importance of early mobility in the ICU (Devlin JW, et al. Crit Care Med. 2018;46[9]:e825). Multiple studies have shown a positive impact of early mobility in the ICU on patients’ outcomes (Tipping CJ, et al. Intensive Care Med. 2017;43:171).
This is especially true for critically ill patients, in which weakness is more common and can result in worse outcomes (Kress JP, et al. N Engl J Med.The recent TEAM study examined an early mobility approach in mechanically ventilated patients and found no difference in the primary outcome of alive and out-of-hospital at 180 days (N Engl J Med. 2022;387:1747).
Before concluding, it is worth realizing that the usual care arm included mobilization that was otherwise normally provided. The intervention arm protocolized the early mobility to be done simultaneously with the minimization of sedation. Patients’ assessment occurred in 81% in the usual care arm vs 94% in the intervention arm; both numbers are much higher than reported data in the ICU (Jolley SE, et al. Crit Care Med. 2017;45:205).
Revisiting the question of early mobility in the ICU, more data are needed to clarify the best methodology, sedation, timing, amount, and type of patients who will benefit the most. Until then, it should remain a goal for ICUs and part of the daily discussion when caring for critically ill patients.
Mohammed J. Al-Jaghbeer, MBBS, FCCP
Section Member-at-Large
Salim Surani, MD, MPH, FCCP
Critical Care Network
Nonrespiratory Critical Care Section
2014;370:1626). This advocacy is endorsed by major societies and guidelines, like the ABCDEF bundle (Balas MC, et al. Crit Care Med. 2013;41:S116), in which “E” stands for Early mobility and exercise. In fact, the PADIS guidelines, addressing Pain, Agitation, Delirium, Immobility, and Sleep in the ICU, added Immobility and Sleep (the “I” and “S” in PADIS) to the prior PAD guidelines in the latest update in 2018, to stress the importance of early mobility in the ICU (Devlin JW, et al. Crit Care Med. 2018;46[9]:e825). Multiple studies have shown a positive impact of early mobility in the ICU on patients’ outcomes (Tipping CJ, et al. Intensive Care Med. 2017;43:171).
This is especially true for critically ill patients, in which weakness is more common and can result in worse outcomes (Kress JP, et al. N Engl J Med.The recent TEAM study examined an early mobility approach in mechanically ventilated patients and found no difference in the primary outcome of alive and out-of-hospital at 180 days (N Engl J Med. 2022;387:1747).
Before concluding, it is worth realizing that the usual care arm included mobilization that was otherwise normally provided. The intervention arm protocolized the early mobility to be done simultaneously with the minimization of sedation. Patients’ assessment occurred in 81% in the usual care arm vs 94% in the intervention arm; both numbers are much higher than reported data in the ICU (Jolley SE, et al. Crit Care Med. 2017;45:205).
Revisiting the question of early mobility in the ICU, more data are needed to clarify the best methodology, sedation, timing, amount, and type of patients who will benefit the most. Until then, it should remain a goal for ICUs and part of the daily discussion when caring for critically ill patients.
Mohammed J. Al-Jaghbeer, MBBS, FCCP
Section Member-at-Large
Salim Surani, MD, MPH, FCCP
Novel therapies for neuromuscular disease: What are the respiratory and sleep implications?
Sleep Medicine Network
Home-Based Mechanical Ventilation & Neuromuscular Disease Section
Novel therapies for neuromuscular disease: What are the respiratory and sleep implications?
The natural history of respiratory impairment in children and adults with progressive neuromuscular disease (NMD) often follows a predictable progression. Muscle weakness leads to sleep-disordered breathing and sleep-related hypoventilation, followed by diurnal hypoventilation, and, ultimately leads to respiratory failure. A number of disease-specific and society guidelines provide protocols for anticipatory respiratory monitoring, such as the role of polysomnography, pulmonary function testing, and respiratory muscle strength testing. They also guide the treatment of respiratory symptoms, such as when to initiate cough augmentation and assisted ventilation.
2021;56[4]:700).
Researchers are also assessing the role of home testing to diagnose hypoventilation (Shi J, et al. Sleep Med. 2023;101:221-7) and using tools like positive airway pressure device data to guide treatment with noninvasive ventilation (Perrem L et al. Pediatr Pulmonol. 2020;55[1]:58-67). While these advances in therapy are exciting, we still do not know what the long-term respiratory function, prognosis, or disease progression may be. Questions remain regarding how to best monitor, and at what frequency to assess, the respiratory status in these patients.
Moshe Y. Prero, MD
Section Member-at-Large
Sleep Medicine Network
Home-Based Mechanical Ventilation & Neuromuscular Disease Section
Novel therapies for neuromuscular disease: What are the respiratory and sleep implications?
The natural history of respiratory impairment in children and adults with progressive neuromuscular disease (NMD) often follows a predictable progression. Muscle weakness leads to sleep-disordered breathing and sleep-related hypoventilation, followed by diurnal hypoventilation, and, ultimately leads to respiratory failure. A number of disease-specific and society guidelines provide protocols for anticipatory respiratory monitoring, such as the role of polysomnography, pulmonary function testing, and respiratory muscle strength testing. They also guide the treatment of respiratory symptoms, such as when to initiate cough augmentation and assisted ventilation.
2021;56[4]:700).
Researchers are also assessing the role of home testing to diagnose hypoventilation (Shi J, et al. Sleep Med. 2023;101:221-7) and using tools like positive airway pressure device data to guide treatment with noninvasive ventilation (Perrem L et al. Pediatr Pulmonol. 2020;55[1]:58-67). While these advances in therapy are exciting, we still do not know what the long-term respiratory function, prognosis, or disease progression may be. Questions remain regarding how to best monitor, and at what frequency to assess, the respiratory status in these patients.
Moshe Y. Prero, MD
Section Member-at-Large
Sleep Medicine Network
Home-Based Mechanical Ventilation & Neuromuscular Disease Section
Novel therapies for neuromuscular disease: What are the respiratory and sleep implications?
The natural history of respiratory impairment in children and adults with progressive neuromuscular disease (NMD) often follows a predictable progression. Muscle weakness leads to sleep-disordered breathing and sleep-related hypoventilation, followed by diurnal hypoventilation, and, ultimately leads to respiratory failure. A number of disease-specific and society guidelines provide protocols for anticipatory respiratory monitoring, such as the role of polysomnography, pulmonary function testing, and respiratory muscle strength testing. They also guide the treatment of respiratory symptoms, such as when to initiate cough augmentation and assisted ventilation.
2021;56[4]:700).
Researchers are also assessing the role of home testing to diagnose hypoventilation (Shi J, et al. Sleep Med. 2023;101:221-7) and using tools like positive airway pressure device data to guide treatment with noninvasive ventilation (Perrem L et al. Pediatr Pulmonol. 2020;55[1]:58-67). While these advances in therapy are exciting, we still do not know what the long-term respiratory function, prognosis, or disease progression may be. Questions remain regarding how to best monitor, and at what frequency to assess, the respiratory status in these patients.
Moshe Y. Prero, MD
Section Member-at-Large