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RESEARCH AT CPC-M - Rare disease: Idiopathic pulmonary fibrosis

RESEARCH AT CPC-M

Rare disease: Idiopathic pulmonary fibrosis

How could the constant deterioration of the disease be slowed down?
Is the lung vitality capacity an indicator?

Although idiopathic pulmonary fibrosis (IPF) is rare, it is not curable. IPF is a lung disease of unknown etiology that is characterized by progressive scarring of the pulmonary parenchyma that leads to decreased lung function capacity and respiratory failure in the course of time. This course of disease might individually occur slower or faster in some patients and up to now it is not predictable how the disease will develop in a single patient. The median term of survival after diagnosis is only about 3-4 years. For a certain subgroup of patients undergoing lung transplantation is the last possible option.

What is known so far: IPF patients often undergo acute, significant deterioration of the disease, so-called exacerbations, accompanied with sudden, increased respiratory distress. However, exacerbations significantly increase the risk of death: most IPF patients die within the following 12 months after such exacerbations.

Illustration lung fibrosis
© Angelika Kramer
Grafic illustration of lung fibrosis

A main issue is therefore: could it improve the life expectancy of IPF patients if exacerbations are detected earlier?

The pilot study AE-IPF aims to answer this question. The study is conducted at four DZL locations, including the DZL Munich site, the CPC-M. The key question: If the patients regularly measure the vital capacity of the lung at home and forward the results to a central location - would it then be possible for the physicians to recognize an exacerbation in time? And would that have a positive effect on the disease progress?

 

Vital capacity (VC) – possible value for the early detection of exacerbations?

The vital capacity (VC) indicates how much volume or how much air the lung can breathe in and out. If this value deteriorates, an accelerated progression of the disease can be expected. In addition, unpredictable exacerbations are increasing, that on the other hand significantly deteriorate the disease - the mortality risk increases. A vicious circle.

Study patient with spirometry device and App
IPF patient with spirometry

Therefore we provide the participants of the AE-IPF pilot study with adequate technology to perform the measurement of all the relevant lung function parameters themselves at home and to transmit the values to the physicians:

 

• A device for spirometry, the measurement of the vital capacity of the lung
• A tablet including the app "My Health Monitor"

The patients then perform measurement of their vital capacity every morning for one year with the spirometry device which then automatically transmits data via Bluetooth to the tablet that then stores all measurement results in an electronical diary. The responsible physicians as well as the study physicians can read the results at any time. In addition, the study participants are provided with a diary and answer a questionnaire on their quality of life every 12 weeks.

Breaking the vicious circle - slowing down the progressive course of IPF

Spirometry device and App
Spirometry device and App

The tasks for the study participants are the following:If the measured values deteriorate by 5% within three days, they should report immediately to the study physicians. To ensure that the flow of information is additionally secured, the study physicians automatically receive an e-mail confirming the deterioration in value. Furthermore, the study team performs up to five check-ups. Here, blood, urine and saliva samples are collected and nasal swabs are taken. These biosamples will be stored in the bioArchive of the CPC-M and enable further research.

The final goal of the researchers: to detect and treat exacerbations faster - which could slow the progression of the disease.

Group leader

[Translate to Englisch:] Prof. Dr. med. Jürgen Behr

Prof. Dr. med. Jürgen Behr

+49 89 4400 75310
direktion.med5@med.uni-muenchen.de

Director Medizinische Klinik und Poliklinik V
LMU Klinikum der Universität München
Marchioninistraße 15

81377 München

Team

Dr. Marion Frankenberger,
Study coordination
Dr. Gabriela Leuschner,
Principal Investigator
Dr. Tobias Veit, Study physician
Patricia Sklarek, Study Nurse
Christina Mihm, MTAF, Study Nurse
Nicole Seeling, MTAF

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