Diagnosed February 2006

To introduce myself, my name is Trevor. I am now 16 years old. I never thought that I would make it to 16, but I am still here. I am an Aussie living in rural Queensland. I have been healthy and fit all of my life, until about September last year. I had limb pains, sort of like rheumatism, but the pain and swelling never really went away, just moved around from joint to joint, elbows, ankles, knees even my hands and shoulders. I also suffered from nose bleeds and didn't really take much notice of that. I went to a ENT specialist about my hearing as I was losing it. They put it down to a middle ear infection and that I should have a grommet put in. My family was organizing that, long waiting lists here in the Health system for any elective surgery. But my limb pain was getting worse, over the time my mum had taken my to quite a few different doctors, they all said it was growing pains, mum wasn't happy with that answer. Early this year (2006) I was bed ridden, with fever and pain. I can remember saying that 'if this is living with growing pains, I don't want to live.' In desperation my mum put me on a commercial flight and took me to Brisbane. After seeing doctors on arrival, I was diagnosed with Wegener's Granulomatosis. That was 9th February 2006.

The rest I don't remember all of it, but here is an outline of what happened.
February 2006 - Respiratory difficulty, bleeding from the lungs, high fever, swollen limbs and associated pain. Hospitalized, started treatment, which included a course of daily Plasmapheresis for 7 days.
March 2006 - still in hospital, developed blood clots in my leg which went to my lungs. So I was put on blood thinners. Kidney function started to deteriate, had a kidney biopsy. Finally got to go home. But on medication of course.
April 2006 - Flew back to Brisbane by Royal Flying Doctor Service with severe gut pain. Next day had surgery where they removed some of my small bowel, due to Wegener's had come back attacking my organs. Recovering from op, treatment started again.
May 2006 From nowhere, cerebral hemorrhage, immense headache, back to HDU. A week later, stroke. No use of arm or leg on left side. Not good. After that I don't remember much, except I was tubed (resporator) for a day or two, but remember having plasmapheresis each day. I also had nearly daily angiograms, to see what was happening and to inject something in there to open up the blood flow. They found another vein at risk of bleeding and after much discussion they gave me the option to have it clipped as it could go anytime or never. What a choice, damned if you do, damned if you don't. I had the nero surgery. Pretty risky stuff. I was worried that I would end up, well not right. But I regained movement and strength in my arm and leg. I eventually got out of ICU, HDU and after about another week, I was let out of hospital to see how I went.
June 2006 So I stayed in the hospital grounds at Ronald McDonald House. I had daily visits for appointments and treatment at the hospital, but I got out a bit in a wheelchair. Went to the movies, spend some money, what I hadn't done in months. After a couple of weeks I was able to go home. But only for 10 days as I had my oncology appointment for my monthly cyclophosmide dose, and doctors visits.
July 2006 Home, great, I needed to go back to work. So I did, I have the best boss, not that the doctors probably like it. But I got so bored, at least at work I see other people. I need to start living life again. Mind you at times I think I may have been better off dying. End of problem. Yes, I get frustrated with the whole thing. Why me? Anyway...
August 2006 Return to hospital oncology appointment for my monthly cyclophosmide dose, and doctors visits. Approx 3-4 days away from home. My mum comes with me of course. I can't remember where and when I have to be anywhere, she does all that. Actually she stayed with me all of the time. Back home, going to work again. I take each day as it comes. Apparently, I am in what they call 'remission'. Some days I get very tired. Next hospital visit mid September.

I have created a BLOG to keep things up to date

Previously I had this on a website, this is the link, but it does not seem to load properly at times, so I was recommended to use this free service. Thanks to Blogspot, this makes it easier for me to enter when I want to say something. I will no longer be updating there. Here is where I will when I can, or feel like. I feel like I am talking to myself. But maybe this will be a good thing to look back on, if not at least I left my footprint on the internet.

I don't know how long I will be around for, so it is easier for me.

What is Wegener's Granulomatosis

Wegener's Granulomatosis is a disease where the blood vessels are inflamed. This is called vasculitis. This inflammation causes damage to the organs of the body by decreasing their blood flow, which destroys their normal tissue. This is called necrosis.

The inflammation damages important organs of the body by limiting blood flow to those organs and destroying normal tissue. The organs afffected most commonly are the lungs and the kidneys.

Wegener's Granulomatosis is a disease that can involve any organ system, but specifically affects the upper and lower respiratory tract (nose, sinuses, windpipe and lungs), kidneys, and small blood vessels (capillaries, venules, arterioles and arteries) of the body.

Incidence:The disease is uncommon occuring in 0.4 per 100 000 people. Peak incidence is in the fifth decade of life, with an average age at diagnosis of 45 years however, the disease can occur in all age groups.

What causes Wegener's Granulomatosis

Wegener's granulomatosis is thought to be an auto-immune disease for which there is no known cause.

Natural History:

The complete syndrome usually quickly progresses to renal failure once the vascular phase begins. Nasal and pulmonary lesions are common in those with limited disease, these individuals may or may not have systemic involvement. These pulmonary lesions can improve or worsen spontaneously.

Prognosis:

If left untreated, 90% of patients will die within 2 years of diagnosis. With corticosteroid and cyclosporin treatment the 5-year-survival is 95%. Causes of death include complications from underlying disease such as myocardial infarction, heart failure, respiratory failure or renal failure or complications from the immunosuppressant treatment given such as infection, malignancy. Other complications include massive hemoptysis, respiratory failure, acute or chronic renal failure, deafness, blindness and neuropathy. Early diagnosis and treatment is crucial in order to improve remission rate and avoid kidney disease and complications.

Investigation:

Blood tests can help determine diagnosis, and may show low red blood cell count (anaemia), and an elevated white blood cell count which is indicative of inflammation somewhere in the body. If the kidneys are involved, the patient's urine can be analysed under a microscope for protein, red blood cells, and structures called red blood cell casts. Blood tests investigating kidney function may be abnormal. Patients may have a blood test that shows the presence of a type of antibody (a disease-fighting protein) called antineutophilic cytoplasmic antibodies. If this is present in the blood, it is highly indicative of the disease. A chest x-ray may be performed if the lungs are involved.

Treatment Overview:

With the appropriate treatment, the outlook is good for patients with Wegener's granulomatosis. [Does not look good to me] Current treatment consists of a combination of a corticosteroid (prednisone) and a cytotoxic agent. Cyclophosphamide is the drug of choice. Azathioprine is less effective but is a good alternative for those patients who may develop side-effects from cyclophosphamide. Methotrexate can also be used in this case. After approximately two to three months of treatment, prednisone dose is slowly reduced so that the patient is on cyclophosphamide only. Cyclophosphamide therapy must continue until greater than one year after clinical remission of the disease. After this an alternative drug such as azathioprine or methotrexate can be used.