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Lederer DJ, Martinez FJ. Idiopathic Pulmonary Fibrosis. N Engl J Med. 2018; 378:1811-1823 DOI: 10.1056/NEJMra1705751
Submitted by Priscilla Robles, BScPT, MSc, PhD
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterized by a chronic, progressive, interstitial fibrosis of unknown cause. Its prevalence is increasing worldwide with incidence in North America and Europe being of 3 to 9 cases/100,000 person-years. This review narrates the current state of understanding of IPF.
Unexplained exertional dyspnea that progresses over a period of months to years, chronic dry cough, velcro-like crackles and low carbon monoxide diffusing capacity are nearly universal findings and should prompt physicians to consider IPF in the differential diagnosis. Pathobiologic features include recurrent epithelial-cell injury, senescent alveolar epithelial cells, profibrotic mediators leading to matrix deposition by myofibroblasts, microbiome changes, and host defense abnormalities. Older age, male sex and cigarette smoking are considered risk factors.
A focused history, physical examination and high-resolution computerized tomography and/or lung biopsy evidencing “usual interstitial pneumonia” (UIP) patterns are obtained to determine the disease presence.
The authors recommend nintedanib and pirfenidone as safe and effective pharmacotherapeuticals to reduce disease progression. Smoking cessation, immunization, titrated supplemental oxygen and pulmonary rehabilitation are shown to improve exercise tolerance and quality of life. Lung transplantation can prolong survival in highly selective candidates.
Approximately 10-20% of patients are at increased risk for acute exacerbation which may be triggered by a clinical event (e.g. infection, aspiration or drug toxicity), but are frequently idiopathic. Deaths mostly occur from progressive, chronic hypoxemic respiratory failure.
This disease carries a poor prognosis with a median survival of 3.8 years among adults 65 years of age and older in the U.S. Palliative care is rarely implemented for patients with IPF before end of life. Thus, continued efforts towards greater disease recognition by primary care providers with earlier involvement of multidisciplinary teams in the diagnosis process, and more preventative and individualized approaches (e.g. screening biomarkers, therapies targeting fibroproliferation) could turn IPF in a lifelong chronic disease in the future.
Owens, O.L, Beer, J.M., Gallerani, D.G., Myhren-Bennett, A.R., & McDonnell, K.K. (2018). JMIR Mhealth Uhealth; 6(5):e124.
Submitted by Julie Duff Cloutier, RN BScN, MSc. Assistant Professor Laurentian University School of Nursing.
With the growing use of mobile technologies, many persons including those living with chronic lung diseases are using the Internet as a resource for health information. However, despite the popularity of health-related mobile applications (apps), there are no regulatory authorities to validate the quality of the health-related content. The authors conducted a systematic review of stress-management apps promoting mindfulness-based strategies in adults with chronic lung disease, available in the Google Play Store and/or the Apple Store. The primary aim was to evaluate to what extent the content was evidence-based. A secondary aim was to evaluate the usability, readability, and cultural sensitivity of the apps. The search yielded 768 apps. Following application of the inclusion criteria, 9 apps were included in this review. None of the reviewed apps fully met the criteria for scientific evidence, however 2 apps partially met the criteria. Most apps partially met criteria for engagement and for being interactive. No apps met the criteria for cultural sensitivity and the average readability was 10th grade. The authors conclude with 5 key recommendations for improving the quality of commercially available apps aimed at those living with chronic respiratory disease.
Ianthe Boden, Elizabeth H Skinner, Laura Browning, Julie Reeve, Lesley Anderson, Cat Hill, Iain K Robertson, David Story, Linda Denehy, BMJ 2018;360:j5916
Submitted by Shirley Quach, HBsc, RRT
Post-operative pulmonary complication (PPC) is a common complication, occurring in 10-50% of patients after upper abdominal surgery. It may include pneumonia and severe atelectasis, which are strongly associated with increased mortality, morbidity and healthcare costs. Breathing exercises may be beneficial in limiting the possibility of PPC, however, there is insufficient evidence to support this hypothesis. Timing may also be of essence, as initiating breathing exercises immediately post-surgery could reduce delay in starting physiotherapy in patients.
Pre-operative education and breathing exercises could benefit patients by minimizing the time to initiating breathing exercises, and therefore minimizing the risks of PPC. A randomized controlled trial consisting of 441 adults receiving major open upper abdominal surgery who were separated into the control group (patients received information booklet pre-operatively) or intervention group (patients received 30 minutes of physiotherapy education and breathing exercise training session pre-operatively). The purpose of this trial was to determine the efficacy of delivering a preoperative physiotherapy session to reduce PPC after upper abdominal surgery.
The incidence of PPC was halved (0.48, CI 0.3-0.75, p=0.001), with an absolute risk reduction of 15% (CI 7-22%) within 14 postoperative hospital days in the intervention group. There are no significant differences in secondary outcomes measured.
There were a few limitations with this study, such as: a need to expand this trial to other sites; level of therapist’s experience may also have influenced effectiveness of educational delivery and outcome.
Despite these limitations, the trial demonstrates that preoperative education and training delivered within 6 weeks of surgery by a physiotherapist could reduce the incidences and risk of PPC within 14 days of surgery. The researchers recognized a need to validate this preoperative education in other elective surgical procedures.