Patients with idiopathic pulmonary fibrosis, an incurable, progressive fibrotic interstitial lung disease, suffer an impaired quality of life due to symptoms, resultant functional limitations, and ...the constraints of supplemental oxygen. Two antifibrotic medications, nintedanib and pirfenidone, are approved for the treatment of idiopathic pulmonary fibrosis. Both medications slow the rate of decline of lung function, but their effect on patient-reported outcomes is not yet fully understood. Nintedanib may slow the decline in health-related quality of life for treated patients. Pirfenidone may slow the progression of dyspnea and improve cough. Patients and providers should participate in shared decision-making when starting antifibrotic therapy, taking into consideration the benefits of treatment in addition to drug-related side effects and dosing schedules. Although antifibrotic therapy may have an impact on health-related quality of life, providers should also focus on comprehensive care of the patient to improve health-related outcomes. This includes a multidisciplinary evaluation, diagnosis and treatment of comorbid medical conditions, and referral to and participation in a pulmonary rehabilitation program. Keywords: interstitial lung disease, idiopathic pulmonary fibrosis, patient-reported outcome measures, quality of life, nintedanib, pirfenidone
Patients with idiopathic pulmonary fibrosis, an incurable, progressive fibrotic interstitial lung disease, suffer an impaired quality of life due to symptoms, resultant functional limitations, and ...the constraints of supplemental oxygen. Two antifibrotic medications, nintedanib and pirfenidone, are approved for the treatment of idiopathic pulmonary fibrosis. Both medications slow the rate of decline of lung function, but their effect on patient-reported outcomes is not yet fully understood. Nintedanib may slow the decline in health-related quality of life for treated patients. Pirfenidone may slow the progression of dyspnea and improve cough. Patients and providers should participate in shared decision-making when starting antifibrotic therapy, taking into consideration the benefits of treatment in addition to drug-related side effects and dosing schedules. Although antifibrotic therapy may have an impact on health-related quality of life, providers should also focus on comprehensive care of the patient to improve health-related outcomes. This includes a multidisciplinary evaluation, diagnosis and treatment of comorbid medical conditions, and referral to and participation in a pulmonary rehabilitation program. Keywords: interstitial lung disease, idiopathic pulmonary fibrosis, patient-reported outcome measures, quality of life, nintedanib, pirfenidone
Management of patients with interstitial lung disease (ILD) requires subspecialized, comprehensive, multidisciplinary care. The Pulmonary Fibrosis Foundation established the Care Center Network (CCN) ...in 2013 with identified criteria to become a designated CCN site. Despite these criteria, the essential components of an ILD clinic remain unknown.
How are ILD clinics within the CCN structured? What are the essential components of an ILD clinic according to ILD physician experts, patients, and caregivers?
This study had three components. First, all 68 CCN sites were surveyed to determine the characteristics of their current ILD clinics. Second, an online, three-round modified Delphi survey was conducted between October and December 2019 with 48 ILD experts participating in total. Items for round 1 were generated using expert interviews. During rounds 1 and 2, experts rated the importance of each item on a 5-point Likert scale. The a priori threshold for consensus was more than 75% of experts rating an item as important or very important. In round 3, experts graded items that met consensus and ranked items deemed essential for an ILD clinic. Third, ILD patient and caregiver focus groups were conducted and analyzed for content to determine their perspectives of an ideal ILD clinic.
Forty items across four categories (members, infrastructure, resources, and multidisciplinary conference) achieved consensus as essential to an ILD clinic. Patient and caregiver focus groups identified three major themes: comprehensive, patient-centered medical care; expanded access to care; and comprehensive support for living and coping with ILD.
The essential components of an ILD clinic are well-aligned between physician experts and patients. Future research can use these findings to evaluate the impact of these components on patient outcomes and to inform best practices for ILD clinics throughout the world.
Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with poor quality of life. Debilitating symptoms and the reality of shortened survival impact patients’ physical and emotional ...well-being and constrain the lives of patients’ caregivers. This study assessed the informational needs of medical providers who care for patients with IPF, IPF patients themselves, and their caregivers. Tailored surveys were sent electronically to providers, patients with IPF, and caregivers of patients with IPF collected on a rolling basis in March of 2017. Providers answered questions regarding their own informational needs and what information they believed patients needed. Patients and caregivers identified their own informational needs and the perceived needs for each other. About 2636 surveys were sent to providers, including 2041 to physicians, of whom 156 completed it. One hundred sixty patients and 29 caregivers responded to the survey via a link on a website. Eighty-six percent of providers described themselves as physicians who diagnose and treat IPF patients themselves. Providers ranked information on “making the diagnosis of IPF” as their top informational need. Patients and caregivers chose “disease progression/what to expect” as the most important informational need for themselves and for each other. Providers want to make a correct diagnosis when IPF is in the differential diagnosis. Patients and caregivers desire clarity around how IPF will behave over time and what their futures with IPF will look like. Resources for patients and their caregivers should include information on disease natural history in empathically worded, clear, and easily accessible formats.
May patients with interstitial lung disease (ILD) require supplementary oxygen (O2) therapy to maintain normoxia. However, ambulatory O2 delivery devices are constraining and cumbersome. The ...physiologic and symptomatic impact of these devices on ILD patients is unknown.
We conducted a prospective study of 30 clinically stable ILD patients (with varying disease severity), half of whom used O2 at baseline. Each subject completed two six-minute walk tests (6MWTs); for O2 users, one walk was completed while wearing a backpack (weight 7.2 pounds) containing a tank with compressed O2, and for non-users, one walk was completed with a similarly-weighted backpack. For each subject, during the second walk, no backpack was worn; for the second walk, O2 users received oxygen via a stationary delivery system. For both walks, O2 non-users wore a portable metabolic system, which measured variables related to respiratory physiology and gas exchange. Borg dyspnea and exertion ratings were recorded after each walk.
Wearing the O2-containing backpack resulted in decreased distance covered during the 6MWT, and increased dyspnea and perceived exertion among O2 users. While wearing the weighted backpack, O2 non-users had a significantly lower peripheral O2 saturation and distance-saturation product. Compared with carrying O2 in the backpack, receiving O2 via the stationary concentrator resulted in the largest improvement in walk distance for the three subjects with greatest impairment at baseline (6MWT ≤ 300 m).
Among ILD patients, carrying portable O2 versus receiving O2 via a stationary concentrator results in significantly greater dyspnea and shorter distances covered in timed testing. Patients with the greatest impairment may be affected most. When prescribing O2, practitioners should alert patients to this effect and help patients decide on the best O2 delivery mode to meet their needs.
•Patients with interstitial lung disease often require supplemental oxygen.•When patients with ILD carry their portable oxygen, they perceive greater dyspnea and walk shorter distances.•Costs associated with carrying oxygen include greater dyspnea and less distance covered.•More functionally impaired patients with ILD appear to be affected most.
Patients with pulmonary fibrosis (PF) have impaired quality of life, and research suggests that dyspnea and physical activity are primary drivers. As PF progresses, some patients notice the disease ..."shrinks their worlds". The objective of this study is to describe movement (both physical activity and activity space) in a cohort of patients with PF of various etiologies who have not been prescribed supplemental oxygen (O
).
Subjects with PF not on supplemental O
during the day were enrolled from across the U.S. from August 2013 to October 2015. At enrollment, each subject completed questionnaires and, for seven consecutive days, wore an accelerometer and GPS tracker.
One hundred ninety-four subjects had a confirmed diagnosis of PF and complete, analyzable GPS data. The cohort was predominantly male (56%), Caucasian (95%) and had idiopathic pulmonary fibrosis (30%) or connective tissue disease related-PF (31%). Subjects walked a median 7497 (interquartile range IQR 5766-9261) steps per day. Steps per day were correlated with symptoms and several quality of life domains. In a model controlling for age, body mass index, wrist- (vs. waist) worn accelerometer and percent predicted diffusing capacity (DLCO%), fatigue (beta coefficient = -51.5 ± 11.7, p < 0.0001) was an independent predictor of steps per day (model R
=0.34).
Patients with PF, who have not been prescribed O
for use during the day, have wide variability in their mobility. Day-to-day physical activity is related to several domains that impact quality of life, but GPS-derived activity space is not. Wearable data collection devices may be used to determine whether and how therapeutic interventions impact movement in PF patients.
NCT01961362 . Registered 9 October, 2013.
A 27-year-old man with OSA, posttraumatic stress disorder, and chronic mechanical back pain presented with a 3-day history of acute atraumatic worsening of his low back pain as well as right groin ...numbness that was exacerbated by walking. He also complained of bilateral leg “heaviness,” pain, and swelling, all becoming so severe that he rented a wheelchair for mobility.
Dyspnea is the hallmark symptom of pulmonary fibrosis. Supplemental oxygen (O
) is prescribed to many patients with pulmonary fibrosis in hopes of alleviating dyspnea and improving physical ...functioning. We used response data from the University of California San Diego Shortness of Breath Questionnaire (UCSD) which was administered monthly in the context of a longitudinal, observational study to plot a rich trajectory for dyspnea over time in patients with pulmonary fibrosis. We used other data from that study to identify clinical predictors of being prescribed O
and to provide additional information for how UCSD scores could be used for clinical purposes.
We used linear mixed-effects models and multivariate Cox proportional hazards to model change in dyspnea scores over time and to identify significant predictors of time-to-O
-prescription among a pool of clinically-meaningful candidate variables. In the longitudinal study, all decisions, including whether or not to prescribe O
, were made by subjects' treating physicians, not members of the research team.
One-hundred ninety-four subjects with pulmonary fibrosis completed more than one UCSD or were prescribed O
at some point during the follow-up period (N = 43). Twenty-eight of the 43 had analyzable, longitudinal data and contribute data to the longitudinal UCSD analyses. All 43 were included in the time-to-O
-prescription analyses. Subjects prescribed O
had more severe dyspnea at enrollment (38.4 ± 19.6 vs. 22.6 ± 18.7, p < 0.0001) and a steeper increase in UCSD scores over time (slope = 1.18 ± 0.53 vs. 0.24 ± 0.09 points per month, p = 0.02) than subjects not prescribed O
. Controlling for baseline UCSD score and FVC%, subjects with a clinical summary diagnosis of idiopathic pulmonary fibrosis (IPF) were far more likely to be prescribed O
than subjects with other forms of pulmonary fibrosis (hazard ratio = 4.85, (2.19, 10.74), p < 0.0001).
Baseline dyspnea and rise in dyspnea over time predict timing of O
prescription. Accounting for disease severity, patients with IPF are more likely than patients with other forms of pulmonary fibrosis to be prescribed O
. UCSD scores provide clinically useful information; frequent administration could yield timely data on changes in disease status in patients with pulmonary fibrosis.
The longitudinal study is registered on ClinicalTrials.gov ( NCT01961362 ). Registered October 9, 2013.
BackgroundIdiopathic pulmonary fibrosis (IPF) is a progressive, incurable lung disease whose intrusive symptoms rob patients of their quality of life. Patients with IPF rely on their caregivers for ...support and assistance in amounts that vary according to patients’ individual circumstances and disease severity. Knowledgeable and well-informed patients and caregivers are best suited to deal with life-altering conditions like IPF.MethodsWe conducted two hour-long focus groups with 13 patients with IPF and 4 caregivers of patients with IPF to better understand their informational needs and in what format such information should be delivered.ResultsPatients discussed the challenges IPF creates in their daily lives. They wanted information on how to live well despite having IPF, practical information on how they could remain active and travel and how they could preserve their quality of life despite living with a life-threatening disease like IPF. Caregivers wanted information on the general aspects of IPF, because it would help them understand what patients were going through. They also wanted specific information on how to give care to a patient with IPF, even when physical care may not be needed (as in earlier phases of the disease). Patients and caregivers both needed efficient information delivery from trustworthy sources, including the healthcare team involved in their care. They considered both spoken and written information valuable, and ease of access was critical.ConclusionThis study provides valuable insight regarding the informational needs of IPF patients and their caregivers. It is hoped that identifying or creating sources of this information, and insuring that patients and caregivers have access to it, will improve well-being for patients with IPF and their caregivers.
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