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CFTR Function and Cigarette Smoke

Scientists are looking at how the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) potentiator, Ivacaftor, might be used in COPD, specifically chronic bronchitis. They found that cigarette smoking reduces CFTR activity in lung tissue, and that exposing the lung cells directly to a CFTR potentiator reverses this effect. While the vast …

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honey

Potential benefits of honey in Cystic Fibrosis Treatment

  Honey has been used for centuries as a food and sweetener but also for its healing properties. Early uses of honey has been noted in ancient civilisations, in countries such as Egypt, Greece, China and Spain. Honey continues to be a popular food today, and a natural product that is widely used for …

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(Cystic Fibrosis Diabetes) First FDA approved artificial pancreas and other 2016 Digital health highlights

The “What We Learnt in Digital Health in 2016” article (by Bertalan Meskó, MD, PhD) takes a look at the successes, failures and challenges in the field of digital health in 2016 and provides an interesting view on some current trends. The first FDA-approved artificial pancreas is mentioned (which may be of interest …

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Gel Caps

Vitamin D Supplements May Help Improve Lung Function in Cystic Fibrosis

This interesting pilot study examined the impact of vitamin D on Cystic Fibrosis patients’ lung function. In addition to the known importance of adequate Vitamin D on bone health, Vitamin D may play an important role in lung function, due to its effects on the immune system. The study also …

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ORKAMBI® (Lumacaftor/Ivacaftor) latest news: Germany, Australia availability

ORKAMBI® reimbursement agreement reached for Germany – December 19, 2016 Vertex Announces German Reimbursement Agreement for ORKAMBI® (Lumacaftor/Ivacaftor), the First Medicine to Treat the Underlying Cause of Cystic Fibrosis in People Ages 12 and Older with Two Copies of the F508del Mutation –(BUSINESS WIRE)–Vertex Pharmaceuticals Incorporated (Nasdaq:VRTX) today announced it has …

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Member post: goals and managing expectations with Cystic Fibrosis

A little story: When I was 12, I participated in a big folk dancing competition through my school. It consisted of several hundred participants and we danced non-stop for hours in front of a selection panel. An elite group of dancers would be chosen to go on a week-long vacation …

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orkambi

Lumacaftor/Ivacaftor longer trial on Heterozygous F508del Cystic Fibrosis Patients

At the end of November, the results of a clinical trial of Lumacaftor/Ivacaftor, also known as Orkambi, in CF patients with one F508Del mutation were published in the Annals of the American Thoracic Society. A past trial for Orkambi in this type of CF patient (heterozygous F508del) lasted 28 days …

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thinkinginsidethebox

Think INSIDE the box for easier inhaled treatments – cystic fibrosis treatment

It’s taken me a long time, but I finally have a ‘system’ that has made inhaled treatments easier to keep up with. Background In childhood, I was very compliant with the CF treatments, as my parents helped me. I didn’t need to think about it; it just happened. In my late …

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3dprotein

Cystic Fibrosis Genetics: First Structural Map of Cystic Fibrosis Protein

First structural map of the cystic fibrosis protein sheds light on how mutations cause disease. This article highlights this recent discovery in cystic fibrosis genetics. Scientists from Rockefeller University have created the first three-dimensional map of the protein responsible for cystic fibrosis, an inherited disease for which there is no …

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photo of Staph bacteria from NIH (public domain)

The Lung Microbiome and Dysbiosis

The “microbiome” is a hot topic in respiratory medicine, with researchers from diverse fields such as chronic obstructive pulmonary disease, bronchiectasis, pulmonary fibrosis, and lung transplantation seeking to use the newly accessible technology of bacterial ribosomal RNA sequencing and related technologies to characterize airway bacterial communities and to determine the …

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