Follow your nose to discover the cause of Wegener's Disease

I read a really interesting article on Med Page Today that theorised that the nose holds the clues to the cause of vasculitis (including Wegener's Granulomatosis).  

Could the cause of Wegener's Granulomatosis be found in the nose?

It starts off with a lot of technical jargon but don't let that put you off; the practical implications of that science are explained later in the article. I'll summarise things here too, to make it even easier.

The research was undertaken by Peter C. Grayson, MD, head of the Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, in Bethesda, Md.

For many people suffering with Wegener's Disease, the first symptoms are experienced in their nose, with nosebleeds and sinus infections very common. This is one of the reasons it can be so difficult to diagnose GPA (granulomatosis with Polyangiitis), because doctor's are treating what they think is a sinus infection rather than the real cause of the problem. Many sufferers then go on to have worse symptoms such as crusting, bleeding and even the destruction of cartilage and bone.

Examining the genes of people with vasculitis to look for a cause is not a new idea, but what Grayson did was new. Instead of taking gene samples from the sufferer's blood, he took the samples from the original source - the nose.

"Increased SERPINA1 expression in nasal mucosa of patients with GPA raises the possibility of a protease-antiprotease imbalance in the nasal environment that could play a causal role in the development of GPA," Grayson and colleagues wrote.

"The study has given us a really nice list of candidate genes and ideas and pathways to pursue in future experiments," Grayson said.

 Peter C. Grayson, MD

Peter C. Grayson, MD

"The observation that some of the genes we detected were related to the innate immune system suggested that there may be interactions going on between the nasal microbiome and immune cells within the nose. That's a really attractive idea for us to follow," he said in an interview.

They also plan to examine gene expression in deeper nasal tissue biopsy samples.

While this is a really interesting development, it is clear that this is not going to lead to an imminent major breakthrough. For one thing the study was very small and that could cloud the results which could be linked to rhinitis, sarcoidosis and other diseases. However, it is good to know that research is continuing into vasculitis and gives me hope that one day the cause, and ultimately a cure, may be found.

The article is called The Nose Holds the Clues in Vasculitis and you can read it here.

Image from M&R Glasgow under Creative Commons licence.

Fishing as therapy for Wegener's Granulomatosis (GPA)

It's not often I read fishing websites for Wegener's Disease stories, but I saw a story on the Bassmaster website recently and I couldn't resist linking to it. Alicia's dad is a keen fisherman and Alicia has a fish tattoo, so the combination of fishing and Wegener's stuck in my mind.

David Cosner has been battling Wegener's Granulomatosis for 8 years, and his favourite form of therapy is catching a 5lb fish!

David was diagnosed at just 17 years old. He was a keen lacrosse player but when the disease attacked his lungs he couldn't cope with that sport physically so he switched his focus to bass fishing. He fishes competitively despite being prone to lung infections. He has undergone over 200 procedures to treat the GPA affecting his lungs, has had a large part of one lung removed and he has even fallen into a coma twice. At some point in the future David believes he will need a lung transplant.

To help pay towards his medical bills David has a Go Fund Me page here.

If you would like to read the full article go to the Bassmaster website here.

Thanks and an apology from Wegener's Disease

First the apology.

I haven't been blogging here anywhere near as much as I would like. In fact, this is my first blog since the end of March.

The main reason for this is that I work as a wedding photographer and we are now moving into wedding season. In the past 4 weeks I have photographed three weddings and an engagement shoot. If anyone is interested, you can take a look at my website here.  

And here is a photograph from a recent wedding.

Katy & Chris walking over the Millennium Bridge on Newcastle Quayside

Unfortunately I have been too busy to blog here and too busy to finish the edits on the print version of my book Alicia: My Battle with Wegener's Disease. I will be busy over the summer, but I hope to get back into the swing of things once the wedding season finishes around September. In the meantime I will check back in here as and when I can.

Despite my absence from posting, my Wegener's Disease Facebook page following has continued to grow as more and more people find out about it. It now has 218 likes as of writing this post. That level of support just amazes me and I am so grateful for everyone who has liked my page. I hope that the page and this website has helped some of those people in some small way.

Thank you!  

Cindy Abbott finishes The Last Great Race

At her third attempt, Cindy Abbott has finished the Iditarod Last Great Race, a 1000 mile dog sled race through the Alaskan wilderness. The race took her over 13 days to complete.

Cindy is the first woman, and only the second person, to complete the race and climb Mount Everest. And all this while suffering from Wegener's Granulomatosis.

 Cindy Abbott finishes the Iditarod while suffering from Wegener's Disease

Cindy Abbott finishes the Iditarod while suffering from Wegener's Disease

Cindy entered the race in 2013 and 2014, but had to scratch (pull out) before the finish due to personal injuries. In one race she carried on for almost 600 miles with a broken pelvis!

While training for her attempt on Mount Everest, Cindy was finally diagnosed with Wegener's Disease, although she had actually been suffering with it for 14 years. She went on to spend 54 days on the mountain and reach the summit.

It just goes to show that a Wegener's Disease diagnosis doesn't mean that your life is over - there is still adventure to be had!

If you would like to know more about Cindy, I wrote about her Ted talk in this blog post. You can also read more about the race on Knom Radio Mission.

Photo: Francesca Fenzi, KNOM