A new year and a new start for Wegener's Disease!

If you're reading this on the Wegener's Disease blog, then you will already have noticed a big change. I have totally overhauled this website for 2015.

I had a number of goals in mind for the redesign.

The main reason was visual. I felt that the old website was a bit plain and boring and I wanted to make it a little bit more visually interesting, fresh and up to date.

The second reason was navigation, especially in the blog area of the site. Most of the new content to the Wegener's Disease website will be posted in the blog section and on the old version of the site it was not possible to search the blog or flag up the more important, useful posts. I have added a sidebar to the blog which includes a search box and links to other recent blog posts. I have also made the main navigation simpler and easier to access. I hope that you will now find it more straightforward to explore the site and find the content that you are looking for.

The final reason was to link to my Twitter and Facebook accounts more closely to the website so that it you will find it easier to interact with me and read my musings on different platforms.

I hope that you like what I have done to the Wegener's Disease website. If so let me know! Similarly, if I have forgotten to add something or I have made an error somewhere, please get in touch.

I look forward to hearing from you!

What would you like me to write about?

I have covered many topics relating to Wegener's Disease on this website and in this blog, as well as in my book, Alicia: My Battle with Wegener's Disease. However, I'm sure that there are many more topics out there that I haven't read about yet. So this is your chance to tell me what topics you are interested in.


It could be a look at a new drug or treatment. I could write about an organisation that helps people with Wegener's Disease. It might be a news story that I have missed or something more generally related to Wegener's like the post I wrote about stress.

So what are you waiting for? Leave me a comment on this blog, tweet me on twitter or leave a message on Facebook and I'll get to work!

Are you a Spoonie? Living with a chronic illness.

As I built this website and started posting about it on Twitter I noticed I have a number of followers referring to themselves as Spoonies. It was an odd term that stuck in my mind, and I didn't understand what it meant. I googled the term and found a website called But You Don't Look Sick

The website was founded by Christine Miserandino and she suffers from Lupus. One day she was talking to a friend about what it was like to live with Lupus and was struggling to help her friend understand what it was like to live with a chronic illness. The analogy she came up with used spoons as a way of conveying the limitations that living with sickness imposes upon you.

You can read the full explanation in Christine's own words here.

Alternatively, you can listen to Christine talk about it in this Youtube video:

I'll post a brief explanation here too. 

Christine and her friend were in a cafe and Christine was trying to convey not just the physical part of the illness but how being ill actually felt. She gathered up 12 spoons from the tables and gave them to her friend. Christine said:

I explained that the difference in being sick and being healthy is having to make choices or to consciously think about things when the rest of the world doesn’t have to. The healthy have the luxury of a life without choices, a gift most people take for granted. 

Most people start the day with unlimited amount of possibilities, and energy to do whatever they desire, especially young people. For the most part, they do not need to worry about the effects of their actions. So for my explanation, I used spoons to convey this point. I wanted something for her to actually hold, for me to then take away, since most people who get sick feel a “loss” of a life they once knew. If I was in control of taking away the spoons, then she would know what it feels like to have someone or something else, in this case Lupus, being in control.

Christine asked her friend to list the tasks of the day and as her friend 'completed' them Christine would take away a spoon. This even included tasks her friend took for granted such as showering and getting dressed. She had used 6 spoons by the time she had gotten to work!

Throughout the day her friend learned to make choices in order to conserve her spoons, until she only had one left. She could only make a meal and eat it, she couldn't wash the pots, do any chores or do something fun. Her friend now understood what it was like to live with a chronic illness.

Christines explains. 

Its hard, the hardest thing I ever had to learn is to slow down, and not do everything. I fight this to this day. I hate feeling left out, having to choose to stay home, or to not get things done that I want to. I wanted her to feel that frustration. I wanted her to understand, that everything everyone else does comes so easy, but for me it is one hundred little jobs in one. I need to think about the weather, my temperature that day, and the whole day’s plans before I can attack any one given thing. When other people can simply do things, I have to attack it and make a plan like I am strategizing a war. It is in that lifestyle, the difference between being sick and healthy. It is the beautiful ability to not think and just do. I miss that freedom. I miss never having to count “spoons”.

I think that is a great way of explaining disability to someone who has no experience of it and I can now understand why people refer to themselves as Spoonies. 

Are you a Spoonie?

Wegener's Disease and Tracheostomy


A tracheostomy is a surgical procedure which creates an opening in the neck, at the front of the windpipe. A tube is inserted into the opening to assist with breathing. This can then be hooked up to an oxygen supply and ventilator as required. 

Fluid that builds up in the trachea can be removed through the opening. 

Why have a tracheostomy?

A tracheostomy will be required when a person cannot breathe normally because of an underlying health condition or a blockage on one of their airways. It will deliver oxygen to the lungs when they are suffering respiratory failure, it can bypass an airway that has become blocked, and can remove fluid that has built up in the upper airway. 

Many conditions can lead to respiratory failure, such as:

  • Being unconscious or in a coma as a result of a severe head injury or a stroke
  • Being paralysed with a serious neck or spinal cord injury
  • A condition that causes extensive damage to the lungs such as cystic fibrosis or pneumonia
  • Or a condition that causes progressive damage to the nervous system such as motor neurone disease or Guillain-Barre syndrome

The airways can become blocked through:

  • Accidentally swallowing something that gets stuck in the windpipe such as a bone
  • An injury, burn, infection or severe allergic reaction that causes the throat to become swollen and narrowed
  • A cancerous tumour that blocks one or more airways such as mouth cancer or thyroid gland cancer
  • Birth defects that cause abnormal narrowing of the airways.

Fluid can build up in the airways when the person:

  • Can’t cough properly because of muscle weakness, paralysis, or chronic pain
  • Has a serious lung infection such as pneumonia
  • Or has an injury that causes the lungs to fill with blood.

Types of tracheostomy

A planned tracheostomy will be performed to help the patient to breathe because of a long term condition such as Wegener’s Granulomatosis or multiple sclerosis, and an emergency tracheostomy will be performed if someone is unable to breathe because of an injury or accident.

Most planned tracheostomy procedures are percutaneous tracheostomies and are carried out under general anaesthetic in an intensive care unit. The surgeon will make a small 2 centimetre long and use an instrument called a dilator to open a hole in the tissue and trachea. The tube is fed through the incision and guided into place with a guidewire.

The planned alternative to a percutaneous tracheostomy is an open tracheostomy. This may be the preferred method for children under 12 years old, if the patient has a tumour affecting the structure of the neck, the patient is overweight and has a very fat neck or there is an infection in the neck.

An open tracheostomy usually takes place in an operating theatre. The patient will typically be under a general anaesthetic. The surgeon cuts into the lower part of the neck, between the Adams apple and the top of the breastbone. The thyroid gland is cut or moved and the muscles parted so that an incision can be made in the wall of the windpipe. The tracheostomy tube can then be inserted through the opening. 

If you are unable to breathe unaided the tracheostomy tube can be attached to a ventilator.

A dressing is usually placed around the wound and then tape or stitches will hold the tube in place. An X-ray can be taken to check that the tube is in the correct position.

An emergency tracheostomy may be needed following trauma if a person’s airway becomes blocked unexpectedly. A local anaesthetic will usually be administered but the procedure will still be very painful.

The paramedic will ensure that the patient is flat on their back and that their neck is exposed by placing a towel or something similar under their shoulders. It’s likely that the patient won’t be able to breathe unaided so they will often be connected to a ventilator.

Living with a tracheostomy

Many people learn to live with a tracheostomy permanently. They have to adapt their eating talking and exercise, and they have to get used to keeping the tube clean and free of blockages.

Vigorous activity should be avoided for the first six weeks following the procedure. While outside it is important to keep the tracheostomy opening clean and dry. It can be covered with a dressing or some loose clothing such as a scarf.

It is very difficult to speak following the procedure. Air normally passes over the vocal cords at the back of the throat when speaking, but after a tracheostomy the air escapes through the tube instead. A speaking valve can be utilised to fix this problem. This attachment to the regular tracheostomy tube closes every time you breathe out, which prevents air escaping and forces it over the vocal cords. It can take some practice to get used to talking with a speaking valve so you may be referred to a speech therapist to improve your ability to communicate.

Tracheostomy tubes need to be cleaned several times per day to stop them getting clogged with mucus or fluid.

Removing the tracheostomy

When the patient can breathe, protect their airway and clear fluids from their trachea unaided, then the tracheostomy tube can be removed. The opening will usually close on its own within a matter of days. If it doesn’t close naturally, it can be closed using stitches.


A tracheostomy is considered to be a low risk procedure but there can sometimes be complications. These include:

  • Like any other surgical procedure there is a chance of blood loss. If the wound does not heal properly then the tube may have to be removed and surgery carried out to stem the bleed.
  • The windpipe can become infected with bacteria which can be treated with antibiotics.
  • Sometimes air can collect around the lungs and can cause them to collapse. This is known as a pneumothorax. A tube can be inserted into the chest to drain the excess air.
  • If the nerves near the windpipe are accidentally damaged you may have trouble speaking or swallowing.
  • In rare cases the windpipe can collapse in on itself, usually because the tracheostomy has been carried out incorrectly. Further surgery will fix the problem.
  • If the airways have become narrowed then the patient may still have breathing difficulties. A stent may then be used to keep the airways open.

Photograph provided by kind permission of Alin S.

How to know when you are having a flare

Wegener's Disease - How to know when you are having a flare

About half of all Wegener’s sufferers will improve with treatment but then go on to relapse (have a ‘flare’) within two years of stopping their treatment. You may have many such flares in the years following your diagnosis which will require a temporary increase in your treatment. So how do you know when you're having a flare? What is a flare?

The most obvious sign is that your original symptoms reoccur. Wegener's Disease attacks people in a number of different ways so everyone's symptoms will be different. Two thirds of Wegener's sufferers have ear, nose or throat problems so this may be an obvious first sign. Are you having headaches. Is your nose blocked or is it running persistently? Do you have lots of mouth or nose ulcers? Are you having nosebleeds? All of these are very common symptoms of a flare.

Fatigue is another good indicator but this can be difficult to detect. Are you tired because you are having a flare or because you have two children and you are working full time? It can be hard to differentiate between the disease and your normal hectic life.

Joint and muscle pain may be your first clue, along with feverish flu-like symptoms.

Your hearing may change. You may experience some deafness or a change in pitch.

While all of these symptoms on their own may point to a flare, perhaps the most surefire way to tell if you are having a flare is that you have two or more of the above symptoms simultaneously. Having a persistently runny nose and joint pain should be a warning sign.  Constant headaches and a loss of hearing in one ear? Go to the doctor and ask them to check if you're flaring.

If you are diagnosed with Wegener's Disease it is highly likely that you will need to visit your doctor regularly for the rest of your life. One of the reasons will be to check your blood. Your doctor will be looking for elevated C-ANCA levels. As many as 90% of Wegener's sufferers having a flare will have high C-ANCA levels. This test is not 100% conclusive on it's own, however. Some people have naturally elevated C-ANCA levels and are not experiencing active Wegener's, while others are having a flare with low C-ANCA levels. So even though it is not a foolproof method of detecting a flare it is still very useful, and may even signal a flare before you start feeling the symptoms.

Even if you have had no previous kidney involvement, your doctors will probably want to keep checking your urine for signs of protein or blood. They will want to catch any flare where the kidneys become affected as early as possible.

Some people flare because their initial inflammation is under control and their doctors are reducing their medication. If you cut your steroid intake too aggressively you may flare. Although you may be feeling much better than you were when you were diagnosed you have to take heed of the medical advice and be careful when reducing your medication. You may start off taking 50mg of prednisolone per day for example and cut that by 5mg to take 45mg. That is a 10% reduction. However, if you are on 10mg per day and cut by 5mg that is a 50% reduction, even though the amount, 5 mg, is the same! A slow steady taper is usually best to reduce flares.

If you are in any doubt whether you are flaring or not, go and see your doctor. Over time you will get better at recognising whether you are flaring or just having a bad day which is unconnected to your GPA. You'll just know.

Image provided by R Nial Bradshaw through Creative Commons Licence.