NETWORKS

Airways Disorders

The asthma COPD overlap syndrome: hype or reality?

In 2014, the Global Initiative for Asthma (GINA) and the Global Initiative for Chronic Obstructive Lung Disease (GOLD) published a joint document on the asthma COPD overlap syndrome (ACOS). While the concept of ACOS is not new, it remains highly debated since its introduction in 1961. ACOS is characterized by persistent airflow limitation with features that are shared with both asthma and COPD. For example, younger asthmatics may develop persistent expiratory airflow limitation from airway remodeling or smoking. Alternatively, patients with COPD may have concurrent features of atopy. Prevalence of ACOS is estimated at 20% in those with obstructive airway disease (Gibson. Thorax. 2015;70[7]:683). Data from the COPDGene cohort suggest that patients with ACOS have more frequent and severe respiratory exacerbations, less emphysema, and greater airway wall thickness (Hardin. Eur Respir J. 2014;44[2]:341).

The mechanisms behind ACOS remain poorly understood. Gelb and colleagues recently reported their observations of loss of lung elastic recoil and presence of centrilobular emphysema in a subset of nonsmokers with chronic asthma and persistent expiratory airflow limitation (Chest. 2015; 148[2]:313; J Allergy Clin Immunol. 2015;136[3]:553). In two COPD cohorts, Christenson and colleagues found that asthma-associated gene signatures were associated with increased disease severity, eosinophil counts, bronchodilator reversibility, and ICS response (Am J Respir Crit Care Med. 2015;191[7]:758). Data presented recently from Spiromics COPD and SARP severe asthma cohorts suggest that subjects with ACOS share a phenotype that falls between COPD and asthma. Allele frequency of candidate genes associated with smoking behavior and allergy in ACOS was intermediate between COPD and asthma (Li et al. Am J Respir Crit Care Med. 2016;A6237).

As with asthma and COPD, there exists significant heterogeneity within ACOS. Despite its high prevalence, evidence on how to consistently identify and best manage this group of patients is lacking. This is in part due to large clinical studies excluding patients with asthma COPD overlap. Additional research will help better understand the different phenotypes and endotypes of ACOS. Real life pragmatic studies evaluating differences in response to therapy are needed to make ACOS more relevant to the practicing clinician.

Dr. Sandhya Khurana, FCCP

Vice-Chair

Clinical Research Network

Airway clearance therapies

Many acute and chronic respiratory conditions such as pneumonia and bronchiectasis, are not only associated with an increase in the quantity and viscosity of respiratory secretions but also with impaired ciliary function and cough, with the latter being very common during mechanical ventilation and after strokes or thoracic surgical procedures. Retention of these secretions are associated with poor patient outcomes, and airway clearance therapies (ACT) are key to improving these outcomes and optimizing recovery after acute illnesses (Kim et. al. Chest. 2011;140[3]:626).

But despite this knowledge, the study of simple ACTs has been largely overlooked for decades. Many of the treatments we currently use, such as positive expiratory pressure devices (for example, the Acapella device), percussive vests, and intrapulmonary percussive ventilation have few studies to support their efficacy, with the ones that exist having very small sample sizes and show no superiority to simple manual techniques (Flume et al. Respir Care. 2009; 54[4]:522). As I walk by a patient’s room and see a respiratory therapist clapping on a patient’s back, I reflect on the fact that research on ACTs has essentially been at a standstill, and we continue to rely on therapies that are no better than the ones used in the 1800s. Our institution has established an Airway Clearance Research Group and has already conducted several bench studies evaluating some of these airway clearance therapies, with goals to develop novel techniques and to start clinical trials, with collaboration from this research steering committee. We hope that furthering the study of ACTs, and determining which therapies are most effective under different clinical settings will not only improve clinical outcomes in chronic conditions such as bronchiectasis but also improve outcomes after pneumonia, strokes, thoracic surgeries, and during mechanical ventilation, where airway clearance is key. Something so simple has the potential to impact a wide population of patients.

Dr. Bravein Amalakuhan

Fellow-in-Training Member

Critical Care

Early mobilization of the critically ill patient

Deconditioning is a well known complication of prolonged ICU stays. It is a factor in long-term mechanical ventilation and contributes to an increased length of stay and comorbidities. The severity of illness, presence of invasive catheters, and the need for sedation in order to provide adequate ventilation are risk factors for immobility. Critically ill patients who are not mobilized experience a decreased quality of life (Needham. JAMA. 2008;300:1685). The changes seen have been shown to persist up to a year after discharge, with a mean loss of 18% body weight, a 5% loss of muscle strength, and a decreased 6-minute walk distance (Herridge et al. N Engl J Med. 2003;348[8]:683; Herridge. Crit Care Med. 2009;37:S457).

There are many safety concerns when mobilizing critically ill patients, but studies have shown that early mobilization is safe with less than 1% of patients having adverse events such as falling, tube removal, and blood pressure instability (Bailey et al. Crit Care Med. 2007;35[1]:139). Barriers to mobilization include ICU staffing, deep sedation, ICU culture, and resources. Adherence to therapy is improved with the use of protocols. These should include automated awakening trials and spontaneous breathing trials that allow for decreases in sedation and, therefore, improvement in mobilization performance (Drolet et al. Phys Ther. 2013;93[2]:197).

Early mobilization improves mortality and decreases morbidity. Safe protocols to implement early mobilization have been developed around the country. A decrease in ICU and hospital length of stay, time supported by mechanical ventilation, and cost reduction with mobilization has been shown in multiple studies. A multidisciplinary team approach and a change in ICU culture will help to accomplish this important initiative.

Dr. Mangala Narasimhan, FCCP

Steering Committee Member

Home-Based Mechanical Ventilation and Neuromuscular Disease

Caregivers and training for kids receiving chronic home invasive ventilation

Despite years of experience in discharging pediatric patients receiving chronic home invasive ventilation, their mortality rate remains high, ranging from 21% to 27.5% with unscheduled readmission at 40% (Boroughs et al. Home Health Nurse. 2012;30:103) to 21% (Edwards et al. J Pediatr. 2010;157[6]:955; Kun et al. Pediatr Pulmonol. 2012:47[4]:409). While there were major improvements in technology and newer ventilators, and better community resources, the one key component of our HMV program remains the same – the caregivers. It is a frightening experience for every family to hear that their child needs ventilator support: every discharge is a daunting task and a life-changing experience.

It seems logical to postulate that we might have improved mortality/readmission outcomes if we have competent caregivers. Recent ATS guidelines recommend that “an awake, trained caregiver should be present at all times, and at least two family caregivers should be trained specifically for the child’s care” (Am J Respir Crit Care Med. 2016;193[8]:e16).The need to shore up on emergency care in the home is further supported when we review studies examining pediatric emergency home ventilation practices for both families and licensed home health nurses (Kun et al. Pediatr Pulmonol. 2010;45[3]:270; Kun. Pediatr Pulmonol. 2015;50[7]:691). Understanding and responding to ventilator alarms remains a major challenge for caregivers and home health nurses. Future directions where we can help our caregivers and families improve home emergency care training include simulation video and using the technology of hand-held devices.

Sheila Kun, RN, BSN

Steering Committee Member

Interstitial and Diffuse Lung Disease

New clinic consortium offers help to patients with rare lung diseases

On the heels of the success of the LAM Foundation’s research and clinic networks, several patient advocacy groups for rare lung diseases approached the LAM Foundation about incorporating the care of other rare lung diseases into the same clinic network.

In 2015, the Rare Lung Diseases Consortium was established. It represents a unique collaboration of these patient advocacy groups, the National Institutes of Health, and clinical investigators. It hopes to utilize the Rare Lung Disease Clinic Network as a resource in understanding the clinical course of several rare lung diseases and as a vehicle to initiate funded clinical trials in patients under the care of clinical investigators working at those various sites.

There are currently 29 geographically distributed Rare Lung Disease Clinic Network clinics in the United States, and another 18 clinics distributed internationally. The clinic directors have held two organizational meetings, including the most recent one in May 2016. The initial three research projects are each noninterventional longitudinal disease observational studies. They include “National Pulmonary Alveolar Proteinosis Registry,” “A Longitudinal Study of Hermansky-Pudlak Syndrome,” and “Multicenter International Durability and Safety of Sirolimus in LAM Trial (MIDAS).”

The first Rare Lung Diseases Consortium Conference is scheduled for September 22-25, 2016, in Cincinnati. It will be a combined educational and research conference, with attendance from clinicians, scientists, patient advocacy organizations, and patients with their families. More information, including a list of the 22 diseases initially designated for care and study in the network and a map of all of the clinic network sites, is available at https://www.rarediseasesnetwork.org/cms/rld/.

Dr. Daniel F. Dilling, FCCP

Steering Committee Member

Airways Disorders

The asthma COPD overlap syndrome: hype or reality?

In 2014, the Global Initiative for Asthma (GINA) and the Global Initiative for Chronic Obstructive Lung Disease (GOLD) published a joint document on the asthma COPD overlap syndrome (ACOS). While the concept of ACOS is not new, it remains highly debated since its introduction in 1961. ACOS is characterized by persistent airflow limitation with features that are shared with both asthma and COPD. For example, younger asthmatics may develop persistent expiratory airflow limitation from airway remodeling or smoking. Alternatively, patients with COPD may have concurrent features of atopy. Prevalence of ACOS is estimated at 20% in those with obstructive airway disease (Gibson. Thorax. 2015;70[7]:683). Data from the COPDGene cohort suggest that patients with ACOS have more frequent and severe respiratory exacerbations, less emphysema, and greater airway wall thickness (Hardin. Eur Respir J. 2014;44[2]:341).

The mechanisms behind ACOS remain poorly understood. Gelb and colleagues recently reported their observations of loss of lung elastic recoil and presence of centrilobular emphysema in a subset of nonsmokers with chronic asthma and persistent expiratory airflow limitation (Chest. 2015; 148[2]:313; J Allergy Clin Immunol. 2015;136[3]:553). In two COPD cohorts, Christenson and colleagues found that asthma-associated gene signatures were associated with increased disease severity, eosinophil counts, bronchodilator reversibility, and ICS response (Am J Respir Crit Care Med. 2015;191[7]:758). Data presented recently from Spiromics COPD and SARP severe asthma cohorts suggest that subjects with ACOS share a phenotype that falls between COPD and asthma. Allele frequency of candidate genes associated with smoking behavior and allergy in ACOS was intermediate between COPD and asthma (Li et al. Am J Respir Crit Care Med. 2016;A6237).

As with asthma and COPD, there exists significant heterogeneity within ACOS. Despite its high prevalence, evidence on how to consistently identify and best manage this group of patients is lacking. This is in part due to large clinical studies excluding patients with asthma COPD overlap. Additional research will help better understand the different phenotypes and endotypes of ACOS. Real life pragmatic studies evaluating differences in response to therapy are needed to make ACOS more relevant to the practicing clinician.

Dr. Sandhya Khurana, FCCP

Vice-Chair

Clinical Research Network

Airway clearance therapies

Many acute and chronic respiratory conditions such as pneumonia and bronchiectasis, are not only associated with an increase in the quantity and viscosity of respiratory secretions but also with impaired ciliary function and cough, with the latter being very common during mechanical ventilation and after strokes or thoracic surgical procedures. Retention of these secretions are associated with poor patient outcomes, and airway clearance therapies (ACT) are key to improving these outcomes and optimizing recovery after acute illnesses (Kim et. al. Chest. 2011;140[3]:626).

But despite this knowledge, the study of simple ACTs has been largely overlooked for decades. Many of the treatments we currently use, such as positive expiratory pressure devices (for example, the Acapella device), percussive vests, and intrapulmonary percussive ventilation have few studies to support their efficacy, with the ones that exist having very small sample sizes and show no superiority to simple manual techniques (Flume et al. Respir Care. 2009; 54[4]:522). As I walk by a patient’s room and see a respiratory therapist clapping on a patient’s back, I reflect on the fact that research on ACTs has essentially been at a standstill, and we continue to rely on therapies that are no better than the ones used in the 1800s. Our institution has established an Airway Clearance Research Group and has already conducted several bench studies evaluating some of these airway clearance therapies, with goals to develop novel techniques and to start clinical trials, with collaboration from this research steering committee. We hope that furthering the study of ACTs, and determining which therapies are most effective under different clinical settings will not only improve clinical outcomes in chronic conditions such as bronchiectasis but also improve outcomes after pneumonia, strokes, thoracic surgeries, and during mechanical ventilation, where airway clearance is key. Something so simple has the potential to impact a wide population of patients.

Dr. Bravein Amalakuhan

Fellow-in-Training Member

Critical Care

Early mobilization of the critically ill patient

Deconditioning is a well known complication of prolonged ICU stays. It is a factor in long-term mechanical ventilation and contributes to an increased length of stay and comorbidities. The severity of illness, presence of invasive catheters, and the need for sedation in order to provide adequate ventilation are risk factors for immobility. Critically ill patients who are not mobilized experience a decreased quality of life (Needham. JAMA. 2008;300:1685). The changes seen have been shown to persist up to a year after discharge, with a mean loss of 18% body weight, a 5% loss of muscle strength, and a decreased 6-minute walk distance (Herridge et al. N Engl J Med. 2003;348[8]:683; Herridge. Crit Care Med. 2009;37:S457).

There are many safety concerns when mobilizing critically ill patients, but studies have shown that early mobilization is safe with less than 1% of patients having adverse events such as falling, tube removal, and blood pressure instability (Bailey et al. Crit Care Med. 2007;35[1]:139). Barriers to mobilization include ICU staffing, deep sedation, ICU culture, and resources. Adherence to therapy is improved with the use of protocols. These should include automated awakening trials and spontaneous breathing trials that allow for decreases in sedation and, therefore, improvement in mobilization performance (Drolet et al. Phys Ther. 2013;93[2]:197).

Early mobilization improves mortality and decreases morbidity. Safe protocols to implement early mobilization have been developed around the country. A decrease in ICU and hospital length of stay, time supported by mechanical ventilation, and cost reduction with mobilization has been shown in multiple studies. A multidisciplinary team approach and a change in ICU culture will help to accomplish this important initiative.

Dr. Mangala Narasimhan, FCCP

Steering Committee Member

Home-Based Mechanical Ventilation and Neuromuscular Disease

Caregivers and training for kids receiving chronic home invasive ventilation

Despite years of experience in discharging pediatric patients receiving chronic home invasive ventilation, their mortality rate remains high, ranging from 21% to 27.5% with unscheduled readmission at 40% (Boroughs et al. Home Health Nurse. 2012;30:103) to 21% (Edwards et al. J Pediatr. 2010;157[6]:955; Kun et al. Pediatr Pulmonol. 2012:47[4]:409). While there were major improvements in technology and newer ventilators, and better community resources, the one key component of our HMV program remains the same – the caregivers. It is a frightening experience for every family to hear that their child needs ventilator support: every discharge is a daunting task and a life-changing experience.

It seems logical to postulate that we might have improved mortality/readmission outcomes if we have competent caregivers. Recent ATS guidelines recommend that “an awake, trained caregiver should be present at all times, and at least two family caregivers should be trained specifically for the child’s care” (Am J Respir Crit Care Med. 2016;193[8]:e16).The need to shore up on emergency care in the home is further supported when we review studies examining pediatric emergency home ventilation practices for both families and licensed home health nurses (Kun et al. Pediatr Pulmonol. 2010;45[3]:270; Kun. Pediatr Pulmonol. 2015;50[7]:691). Understanding and responding to ventilator alarms remains a major challenge for caregivers and home health nurses. Future directions where we can help our caregivers and families improve home emergency care training include simulation video and using the technology of hand-held devices.

Sheila Kun, RN, BSN

Steering Committee Member

Interstitial and Diffuse Lung Disease

New clinic consortium offers help to patients with rare lung diseases

On the heels of the success of the LAM Foundation’s research and clinic networks, several patient advocacy groups for rare lung diseases approached the LAM Foundation about incorporating the care of other rare lung diseases into the same clinic network.

In 2015, the Rare Lung Diseases Consortium was established. It represents a unique collaboration of these patient advocacy groups, the National Institutes of Health, and clinical investigators. It hopes to utilize the Rare Lung Disease Clinic Network as a resource in understanding the clinical course of several rare lung diseases and as a vehicle to initiate funded clinical trials in patients under the care of clinical investigators working at those various sites.

There are currently 29 geographically distributed Rare Lung Disease Clinic Network clinics in the United States, and another 18 clinics distributed internationally. The clinic directors have held two organizational meetings, including the most recent one in May 2016. The initial three research projects are each noninterventional longitudinal disease observational studies. They include “National Pulmonary Alveolar Proteinosis Registry,” “A Longitudinal Study of Hermansky-Pudlak Syndrome,” and “Multicenter International Durability and Safety of Sirolimus in LAM Trial (MIDAS).”

The first Rare Lung Diseases Consortium Conference is scheduled for September 22-25, 2016, in Cincinnati. It will be a combined educational and research conference, with attendance from clinicians, scientists, patient advocacy organizations, and patients with their families. More information, including a list of the 22 diseases initially designated for care and study in the network and a map of all of the clinic network sites, is available at https://www.rarediseasesnetwork.org/cms/rld/.

Dr. Daniel F. Dilling, FCCP

Steering Committee Member

Airways Disorders

The asthma COPD overlap syndrome: hype or reality?

In 2014, the Global Initiative for Asthma (GINA) and the Global Initiative for Chronic Obstructive Lung Disease (GOLD) published a joint document on the asthma COPD overlap syndrome (ACOS). While the concept of ACOS is not new, it remains highly debated since its introduction in 1961. ACOS is characterized by persistent airflow limitation with features that are shared with both asthma and COPD. For example, younger asthmatics may develop persistent expiratory airflow limitation from airway remodeling or smoking. Alternatively, patients with COPD may have concurrent features of atopy. Prevalence of ACOS is estimated at 20% in those with obstructive airway disease (Gibson. Thorax. 2015;70[7]:683). Data from the COPDGene cohort suggest that patients with ACOS have more frequent and severe respiratory exacerbations, less emphysema, and greater airway wall thickness (Hardin. Eur Respir J. 2014;44[2]:341).

The mechanisms behind ACOS remain poorly understood. Gelb and colleagues recently reported their observations of loss of lung elastic recoil and presence of centrilobular emphysema in a subset of nonsmokers with chronic asthma and persistent expiratory airflow limitation (Chest. 2015; 148[2]:313; J Allergy Clin Immunol. 2015;136[3]:553). In two COPD cohorts, Christenson and colleagues found that asthma-associated gene signatures were associated with increased disease severity, eosinophil counts, bronchodilator reversibility, and ICS response (Am J Respir Crit Care Med. 2015;191[7]:758). Data presented recently from Spiromics COPD and SARP severe asthma cohorts suggest that subjects with ACOS share a phenotype that falls between COPD and asthma. Allele frequency of candidate genes associated with smoking behavior and allergy in ACOS was intermediate between COPD and asthma (Li et al. Am J Respir Crit Care Med. 2016;A6237).

As with asthma and COPD, there exists significant heterogeneity within ACOS. Despite its high prevalence, evidence on how to consistently identify and best manage this group of patients is lacking. This is in part due to large clinical studies excluding patients with asthma COPD overlap. Additional research will help better understand the different phenotypes and endotypes of ACOS. Real life pragmatic studies evaluating differences in response to therapy are needed to make ACOS more relevant to the practicing clinician.

Dr. Sandhya Khurana, FCCP

Vice-Chair

Clinical Research Network

Airway clearance therapies

Many acute and chronic respiratory conditions such as pneumonia and bronchiectasis, are not only associated with an increase in the quantity and viscosity of respiratory secretions but also with impaired ciliary function and cough, with the latter being very common during mechanical ventilation and after strokes or thoracic surgical procedures. Retention of these secretions are associated with poor patient outcomes, and airway clearance therapies (ACT) are key to improving these outcomes and optimizing recovery after acute illnesses (Kim et. al. Chest. 2011;140[3]:626).

But despite this knowledge, the study of simple ACTs has been largely overlooked for decades. Many of the treatments we currently use, such as positive expiratory pressure devices (for example, the Acapella device), percussive vests, and intrapulmonary percussive ventilation have few studies to support their efficacy, with the ones that exist having very small sample sizes and show no superiority to simple manual techniques (Flume et al. Respir Care. 2009; 54[4]:522). As I walk by a patient’s room and see a respiratory therapist clapping on a patient’s back, I reflect on the fact that research on ACTs has essentially been at a standstill, and we continue to rely on therapies that are no better than the ones used in the 1800s. Our institution has established an Airway Clearance Research Group and has already conducted several bench studies evaluating some of these airway clearance therapies, with goals to develop novel techniques and to start clinical trials, with collaboration from this research steering committee. We hope that furthering the study of ACTs, and determining which therapies are most effective under different clinical settings will not only improve clinical outcomes in chronic conditions such as bronchiectasis but also improve outcomes after pneumonia, strokes, thoracic surgeries, and during mechanical ventilation, where airway clearance is key. Something so simple has the potential to impact a wide population of patients.

Dr. Bravein Amalakuhan

Fellow-in-Training Member

Critical Care

Early mobilization of the critically ill patient

Deconditioning is a well known complication of prolonged ICU stays. It is a factor in long-term mechanical ventilation and contributes to an increased length of stay and comorbidities. The severity of illness, presence of invasive catheters, and the need for sedation in order to provide adequate ventilation are risk factors for immobility. Critically ill patients who are not mobilized experience a decreased quality of life (Needham. JAMA. 2008;300:1685). The changes seen have been shown to persist up to a year after discharge, with a mean loss of 18% body weight, a 5% loss of muscle strength, and a decreased 6-minute walk distance (Herridge et al. N Engl J Med. 2003;348[8]:683; Herridge. Crit Care Med. 2009;37:S457).

There are many safety concerns when mobilizing critically ill patients, but studies have shown that early mobilization is safe with less than 1% of patients having adverse events such as falling, tube removal, and blood pressure instability (Bailey et al. Crit Care Med. 2007;35[1]:139). Barriers to mobilization include ICU staffing, deep sedation, ICU culture, and resources. Adherence to therapy is improved with the use of protocols. These should include automated awakening trials and spontaneous breathing trials that allow for decreases in sedation and, therefore, improvement in mobilization performance (Drolet et al. Phys Ther. 2013;93[2]:197).

Early mobilization improves mortality and decreases morbidity. Safe protocols to implement early mobilization have been developed around the country. A decrease in ICU and hospital length of stay, time supported by mechanical ventilation, and cost reduction with mobilization has been shown in multiple studies. A multidisciplinary team approach and a change in ICU culture will help to accomplish this important initiative.

Dr. Mangala Narasimhan, FCCP

Steering Committee Member

Home-Based Mechanical Ventilation and Neuromuscular Disease

Caregivers and training for kids receiving chronic home invasive ventilation

Despite years of experience in discharging pediatric patients receiving chronic home invasive ventilation, their mortality rate remains high, ranging from 21% to 27.5% with unscheduled readmission at 40% (Boroughs et al. Home Health Nurse. 2012;30:103) to 21% (Edwards et al. J Pediatr. 2010;157[6]:955; Kun et al. Pediatr Pulmonol. 2012:47[4]:409). While there were major improvements in technology and newer ventilators, and better community resources, the one key component of our HMV program remains the same – the caregivers. It is a frightening experience for every family to hear that their child needs ventilator support: every discharge is a daunting task and a life-changing experience.

It seems logical to postulate that we might have improved mortality/readmission outcomes if we have competent caregivers. Recent ATS guidelines recommend that “an awake, trained caregiver should be present at all times, and at least two family caregivers should be trained specifically for the child’s care” (Am J Respir Crit Care Med. 2016;193[8]:e16).The need to shore up on emergency care in the home is further supported when we review studies examining pediatric emergency home ventilation practices for both families and licensed home health nurses (Kun et al. Pediatr Pulmonol. 2010;45[3]:270; Kun. Pediatr Pulmonol. 2015;50[7]:691). Understanding and responding to ventilator alarms remains a major challenge for caregivers and home health nurses. Future directions where we can help our caregivers and families improve home emergency care training include simulation video and using the technology of hand-held devices.

Sheila Kun, RN, BSN

Steering Committee Member

Interstitial and Diffuse Lung Disease

New clinic consortium offers help to patients with rare lung diseases

On the heels of the success of the LAM Foundation’s research and clinic networks, several patient advocacy groups for rare lung diseases approached the LAM Foundation about incorporating the care of other rare lung diseases into the same clinic network.

In 2015, the Rare Lung Diseases Consortium was established. It represents a unique collaboration of these patient advocacy groups, the National Institutes of Health, and clinical investigators. It hopes to utilize the Rare Lung Disease Clinic Network as a resource in understanding the clinical course of several rare lung diseases and as a vehicle to initiate funded clinical trials in patients under the care of clinical investigators working at those various sites.

There are currently 29 geographically distributed Rare Lung Disease Clinic Network clinics in the United States, and another 18 clinics distributed internationally. The clinic directors have held two organizational meetings, including the most recent one in May 2016. The initial three research projects are each noninterventional longitudinal disease observational studies. They include “National Pulmonary Alveolar Proteinosis Registry,” “A Longitudinal Study of Hermansky-Pudlak Syndrome,” and “Multicenter International Durability and Safety of Sirolimus in LAM Trial (MIDAS).”

The first Rare Lung Diseases Consortium Conference is scheduled for September 22-25, 2016, in Cincinnati. It will be a combined educational and research conference, with attendance from clinicians, scientists, patient advocacy organizations, and patients with their families. More information, including a list of the 22 diseases initially designated for care and study in the network and a map of all of the clinic network sites, is available at https://www.rarediseasesnetwork.org/cms/rld/.

Dr. Daniel F. Dilling, FCCP

Steering Committee Member