Provider and Caregiver Connection™: Improving Outcomes in Idiopathic Pulmonary Fibrosis (IPF) Through a Patient-Centered, Team-Based Approach

 


 

Activity Overview

Idiopathic Pulmonary Fibrosis (IPF) is a rare and progressive fibrotic lung disease with a median survival of 2 ½ to 5 years, which is worse than most cancers. With nonspecific signs and symptoms including dyspnea and cough, diagnosis is challenging and often delayed.  While there is no cure, antifibrotic therapies can slow or halt disease progression and, along with supportive measures, improve patient’s quality of life. 
 
During this Patient Caregiver Connection® Medical Crossfire®, a multidisciplinary panel of expert faculty will share their insights on early, differential diagnosis and discuss clinical practice guideline updates which include conditional recommendations for the antifibrotic therapies pirfenidone and nintedanib. Faculty will then elaborate on the need for a multidisciplinary ‘ABCDE’ approach to address not only the range of co-morbidities associated with IPF but to provide comfort care that assesses and focuses on individual patient needs.  This activity is enhanced with a patient’s own story and perspective, from delays in diagnosis to daily challenges and advice for anyone burdened with a diagnosis of IPF.

Acknowledgement of Commercial Support

This activity is supported by an educational grant from Boehringer Ingelheim Pharmaceuticals, Inc.
 

Instructions for This Activity and Receiving Credit

  • You will need to login to participate in the activity.
  • Each presentation may contain an interactive question(s). You may move forward through the presentation; however, you may not go back to change answers or review videos/content until you finish the presentation.
  • At the end of the activity, “educational content/video files” will be available for your reference.
  • In order to receive a CME/CE certificate, participants must complete the activity.
  • Complete the posttest and pass with a score of 70% or higher, complete the evaluation and then click on request for credit. Participants may immediately download a CME/CE certificate upon completion of these steps.


Target Audience

This CME activity is intended for IPF specialists, primary care physicians, physician assistants, nurse practitioners, nurses and other clinicians who treat patients with IPF.

Learning Objectives

Upon completion of this CME activity, you should be better prepared to:

  • List strategies for confirming an IPF diagnosis, including the utility of high-resolution computed tomography and surgical lung biopsy
  • Distinguish idiopathic from non-idiopathic forms by applying appropriate diagnostic testing, such as high-resolution CT scanning, to characterize distribution of fibrosis and inflammation
  • Apply current guidelines and new clinical evidence to develop a patient-centered, team approach plan for patients with IPF
  • Discuss new evidence and the status of emerging treatments for patients with IPF
  • Describe strategies to engage patients and facilitate a multidisciplinary approach to the management of IPF and associated comorbidities

Faculty, Staff, and Planners' Disclosures

Stephen Brunton, MD
Executive Vice President
Primary Care Education Consortium
Clinical Family Practice
Glendale and Downey, California
Disclosure: No relevant financial relationships with commercial interests.
 
Louis Kuritzky, MD
Clinical Assistant Professor Emeritus
University of Florida
Gainesville, Florida
Disclosure: No relevant financial relationships with commercial interests.
 
Steven Nathan, MD
Medical Director, Advanced Lung Disease and Lung Transplant Program
Professor of Medicine
Virginia Commonwealth University-Inova
Falls Church, Virginia
Disclosure: Grant/research support, consultant, member of speakers bureau: Boehringer Ingelheim, Inc. and Roche-Genentech.
 
Mary M. Salvatore, MD, MBA
Associate Professor of Radiology
Mount Sinai
New York, New York
Disclosure: member of speakers bureau: Genentech, Boehringer Ingelheim Inc. and Rockpointe.

The staff of PER® have no relevant financial relationships with commercial interests to disclose.

Disclosure Policy and Resolution of Conflicts of Interest (COI)

As a sponsor accredited by the ACCME, it is the policy of PER® to ensure fair balance, independence, objectivity, and scientific rigor in all of its CME/CE activities. In compliance with ACCME guidelines, PER® requires everyone who is in a position to control the content of a CME/CE activity to disclose all relevant financial relationships with commercial interests. The ACCME defines “relevant financial relationships” as financial relationships in any amount occurring within the past 12 months that creates a COI.

Additionally, PER® is required by ACCME to resolve all COI. PER® has identified and resolved all COI prior to the start of this activity by using a multistep process.

Off-Label Disclosure and Disclaimer

This activity may or may not discuss investigational, unapproved, or off-label uses of drugs. Participants are advised to consult prescribing information for any products discussed. The information provided in this CME activity is for continuing medical education purposes only and is not meant to substitute for the independent medical judgment of a physician relative to the diagnostic, treatment, and management options for a specific patient’s medical condition.
 
The opinions expressed in the content are solely those of the individual faculty members and do not reflect those of Physicians’ Education Resource®, LLC, or any of the companies that provided commercial support for this activity.

PER Pulse™ Recaps

1 of 3
Improving Outcomes in Idiopathic Pulmonary Fibrosis (IPF) Through a Patient-Centered, Team-Based Approach

The online Provider and Caregiver Connection Improving Outcomes in Idiopathic Pulmonary Fibrosis (IPF) Through a Patient-Centered, Team-Based Approach provides IPF specialists, primary care physicians, physician assistants, nurse practitioners, nurses, and other clinicians who treat patients with IPF guidance on the early diagnosis and evidence-based treatment of the disease.

A multidisciplinary panel answered key questions, supported by the presentation of clinical science, about IPF. This first of 3 PER Pulse Recaps from this program focuses on distinguishing features of IPF and opportunities for early diagnosis.

Steven Nathan, MD, medical director of Inova Advanced Lung Disease and Lung Transplant Program and professor of medicine at Virginia Commonwealth University-Inova, both in Falls Church, Virginia, discussed distinct symptoms patients may present with that suggest a diagnosis of IPF, as well as the necessary steps to take to confirm diagnosis and rule out alternative causes.

  • Idiopathic pulmonary fibrosis typically presents in the sixth or seventh decade with nonspecific symptoms. Common findings include bibasilar inspiratory crackles and, occasionally, finger clubbing. Nonetheless, early diagnosis is more critical than ever given the availability of antifibrotics, which, although not a cure, can slow disease progression.
  • Making the diagnosis of IPF requires a comprehensive clinical evaluation. The differential diagnosis is diverse, including neoplastic conditions; autoimmune conditions, particularly connective tissue diseases; drug toxicity; inhalation of asbestos, silica, and other toxins; and infections, such as cytomegalovirus, pneumonia, and pneumocystis. About two-thirds to three-fourths of idiopathic interstitial pneumonias are IPF. The clinical workup should include testing for autoimmune disease or vasculitis, a prednisone trial to rule out autoimmune disease, and a chest x-ray. Other tests include complete pulmonary function testing, spirometry, lung volumes, and diffusing capacity for carbon monoxide.
  • The pivotal study is a high-resolution CT (HRCT). The most common protocol used is a volumetric imaging of thin sections (usually 1-1.5 mm).1 Guidelines from the American Thoracic Society for the radiographic diagnosis of IPF define a usual interstitial pneumonia (UIP) pattern as fibrosis primarily seen in the lower lobes along the periphery of the lung and honeycombing, although by the time honeycombing is visible, the disease tends to be advanced. Without the honeycombing, this is called a possible UIP pattern or inconsistent with UIP and is more likely to require a lung biopsy for a confirmatory diagnosis.

The current recommendation for a lung biopsy, based on expert opinion and low-quality evidence, is to procure tissue samples from more than 1 site in the lung. Video-assisted thoracic surgery is typically preferred. However, the use of lung biopsy is limited given the age of patients and comorbidities.

Reference

  1. Gruden JF. CT in Idiopathic Pulmonary Fibrosis: Diagnosis and Beyond. AJR Am J Roentgenol. 2016;206(3):495-507. doi: 10.2214/AJR.15.15674

2 of 3
Improving Outcomes in Idiopathic Pulmonary Fibrosis (IPF) Through a Patient-Centered, Team-Based Approach

The online Provider and Caregiver Connection, Improving Outcomes in Idiopathic Pulmonary Fibrosis (IPF) Through a Patient-Centered, Team-Based Approach, provides IPF specialists, primary care physicians, physician assistants, nurse practitioners, nurses, and other clinicians who treat patients with guidance on the early diagnosis and evidence-based treatment of the disease.

A multidisciplinary panel answered key questions, supported by the presentation of clinical science, about IPF. This second of 3 PER Pulse Recaps from this program focuses on the antifibrotics pirfenidone and nintedanib.

  • Pirfenidone and nintedanib delay disease progression. It is difficult to compare one against the other, so they are considered equal, with the panelists noting they use both in their practices. The panelists also noted that every patient with IPF, if asymptomatic with normal lung function, is a candidate for the therapy.
  • However, patients may not notice any difference in their symptoms. This should be an important part of a discussion with the patient, said Steven Nathan, MD, medical director of Inova Advanced Lung Disease and Lung Transplant Program and professor of medicine at Virginia Commonwealth University-Inova, both in Falls Church, Virginia. Patients should realize that taking the medication as directed “is an investment in the future of maintaining their lung function,” he said. If they don’t understand this, they are more likely to be nonadherent.
  • Pirfenidone was assessed in 3 major trials, 2 capacity studies, and the ASCEND study. The ASCEND study included more than 1250 patients. Emerging post hoc data suggest a mortality benefit over time.
  • Nintedanib was assessed in 3 studies, and all demonstrated a delay in disease progression based on forced vital capacity. There was also a reduced risk of acute exacerbations. Follow-up data also suggested some mortality benefit.
  • Adverse effects include nausea, vomiting, diarrhea, and abdominal pain. About 60% of patients on nintedanib will experience diarrhea, so it’s a good idea to prescribe an antidiarrheal with the drug. There is also the risk of photosensitivity rash with pirfenidone, so patients should be counseled about sun exposure. In rare cases, both drugs can lead to abnormal liver function tests, so a baseline and then regular lung function tests should be performed.
  • Clinicians should choose the medication patients are most likely to take as directed.

3 of 3
Improving Outcomes in Idiopathic Pulmonary Fibrosis (IPF) Through a Patient-Centered, Team-Based Approach

The online Provider and Caregiver Connection, Improving Outcomes in Idiopathic Pulmonary Fibrosis (IPF) Through a Patient-Centered, Team-Based Approach, provides IPF specialists, primary care physicians, physician assistants, nurse practitioners, nurses, and other clinicians who treat patients with IPF guidance on the early diagnosis and evidence-based treatment of the disease.

A multidisciplinary panel answered key questions, supported by the presentation of clinical science, about IPF. This third of 3 PER Pulse Recaps focuses on supportive care for those with IPF.

  • Idiopathic pulmonary fibrosis has a significantly negative effect on patient quality of life, leading to depression, feelings of isolation, and anxiety, among other issues. “I tell my patients that I don’t want the disease running or ruining their lives,” said Steven Nathan, MD, medical director of Inova Advanced Lung Disease and Lung Transplant Program and professor of medicine at Virginia Commonwealth University-Inova, both in Falls Church, Virginia. He encourages patients to do everything that’s important to them, including travel.
  • It is also important that patients receive quality preventive care, including vaccinations and infection prevention. Patients often have comorbidities, including sleep apnea and gastroesophageal reflux disease, that must be addressed.
  • Some patients may qualify for lung transplant. They should be evaluated and, if appropriate, placed on the transplant list, even if asymptomatic, in case their condition suddenly deteriorates.
  • If a patient’s condition cannot be managed on supplemental oxygen, Dr. Nathan does not recommend mechanical ventilation unless they are a transplant candidate. At that point, the prognosis is quite poor.
  • Mary Salvatore, MD, associate professor of radiology at Columbia University Irving Medical Center, New York, New York, described bimonthly support groups for patients at which speakers discuss topics such as rehabilitation, oxygen therapy, and radiology. “What’s most helpful to the patients is talking to their peers and seeing how they cope with the disease,” she said.
  • Louis Kuritzky, MD, clinical assistant professor emeritus at the University of Florida in Gainesville, Florida, discussed the ABCDE acronym as an important component of patient-centered care in IPF. It involves assessing the patient’s needs, including any adverse effects from medication; answering their questions; discussing comorbidities; advocating for the patient; setting realistic expectations, particularly about medications; and addressing end-of-life issues. “People are shy or hesitant to bring end-of-life issues up,” Dr. Kuritzky said, “but for these patients, it's a reality.”

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