Weggies Unite: Other Weggie Stories

Here are some Weggies stories from some of my Weggie friends. These are stories submitted for our book project, but that seems to be taking longer than I wanted (due to financial and time constraints) so I have decided to make them available for people to see (with the owner's permission of course - other than Jack who has since passed on.) So with that I will start with Jack's story. Jack was an inspiration and a moral and medical compass for so many sufferers of Wegener's Granulomatosis. He's an amazing spirit and his body and his sagely voice were sadly taken away from us due to complications from the disease and the treatment in late 2011. It has never saddened me that much to loose a friend who I've never met in person in my life. This disease has brought out things in me and I'm sure in others that we had no idea lived inside of us... Rest in peace Jack, you will always be in my heart :

Jack.

Diagnosed 1988 (first symptoms 1986).

32 years old at diagnosis

Living in Birmingham, England when diagnosed.

Areas affected - Sinuses. Hand, knee and hip joints. Skin on hands. Eyes.

Live in Birmingham, attend Queen Elizabeth Hospital Birmingham.

I was born and grew up in a small market town in the middle of England where I went to the local Grammar school. I had a happy home life with my parents and older sisters and brother and also met the girl who I was later to marry. I then moved to the area of Coventry and Birmingham where I went to college and started work as an engineer in the motor manufacturing industry. My girl friend moved in with me and shortly afterwards we bought a house and were married.

I was getting along very well at work and was given a job that involved working by the coast in Southern Italy performing high speed tests on cars at the local test track. I had my immunisation shots and off I went! The work was good fun and my free time was spent swimming in the bay and relaxing in the local cafes all expenses paid. It was not until my last day that I woke up in the night with a splitting head ache and started to wonder what I drank to have such an effect, but it did not get any better and the trip home was not good.

The head ache and sinus pain became increasingly worse until I could no longer sleep. I was being treated with antibiotics with no improvement and soon began a series of minor surgeries to flush my sinus cavities. This culminated in a more major operation called a Bilateral Caldwell Luc after which things seemed to improve to a tolerable level. During this time, I also began to suffer from a variety of skin problems, joint pain and blood shot eyes, but these symptoms subsided too.

Around a year later, it all went horribly wrong again with the return of all symptoms at an elevated level. I was immediately readmitted to the ENT ward, but they were at a loss as to what was wrong with me. I stayed on the ward for two weeks, slowly getting worse while they wondered what to do with me. By this time, I could hardly walk. It was only then that a Locum doctor said that he thought he knew what the problem was because he had just read an article in a journal describing similar symptoms. Tests were made and it was discovered that I probably had Wegener’s Granulomatosis, but I was also in an advanced state of renal failure and general organ shut down problems. They immediately transferred me to the Rheumatology department of the City’s teaching hospital where they ran a Vasculitis Research Unit and I was finally diagnosed and began to receive treatment.

Because of my advanced state of decline, they hit the disease hard with high doses of Cyclophosphamide, Prednisolone and several rounds of Plasmapheresis. Within days, I was feeling fine again, but this was an illusion brought about by the steroids and I remained in hospital for another couple of months while they stabilised my kidney function and reduced the medication to a more sustainable level. I then returned home, but was a frequent visitor to the hospital due to flare ups of Wegener’s, decline of the remaining kidney function and moving on to dialysis. The final step on the road was when I received a renal transplant from a cadaver donor. This was the best gift that anyone could ever give and marked the start of my new life in remission. This was around five years after the start of all the problems and it had been a very difficult time in many ways.

The next 15 years were spent in a condition nearing normality or at least nothing too bad to cope with. I had some bad times when my wife left me and when minor setbacks had me seeking medical attention, but some good times when my wife returned and we started our family of two daughters. One by adoption and one with the help of A.I. Work was going well and I put some effort into building a pension and getting my financial commitments to a minimum. I could see that the good times were not going to last forever since each problem set me back just a little bit more. Most of these were due to drug side effects, but others such as lung involvement were probably Wegener’s related. Eventually, the accumulation of medical conditions forced me to take early retirement from work, but I considered that I had done well to make it this far.

The last five years have not been so kind to my body. Long term use of steroids has resulted in muscle atrophy and Osteoporosis. I have become malnourished due to problems with my digestive system and with swallowing. Multiple fractures and tendon problems have almost stopped me from walking more than a few steps, but life goes on presenting problems to be solved and keeps me from being bored. The transplanted kidney is doing fine and I can still think straight. I can see that many of the pressures associated with modern life are not that important and I enjoy bringing up my family and watching them make their way in the world. The description of “good days and bad days” fits my current situation. On good days my spirits are raised and I think I am making progress towards something more like a normal life again. On bad days, I seriously start to wonder if this is how it ends.

In my own case, continuing to take drugs at quite high doses has been unavoidable. I need them to maintain my transplant and also my Wegener’s is quite aggressive and seems to want to come back as soon as I back off my protection. Every case is very different and for most this will not be the case and drug free remission will be a realistic prospect even if it may be broken by periods of relapse. Knowledge of the disease and drugs to treat it are also slowly improving, although they may never get the sort of attention devoted to Cancer. However, there is still a great deal of ignorance of the disease and its treatment amongst many doctors. I was lucky enough to be seen immediately at a centre specialising in vasculitis research especially involving renal problems. I could not have found a better place to be treated. I am sure that many of the problems with treatment and even deaths are due to poor standards of treatment and lack of experience on the part of the doctor in charge.

Next you'll read Nicole's story ...

My name is Nicole. I was diagnosed with Wegener’s in December 2010 when I was 26 years old. I have lived in Southern California my whole life, and currently reside in Anaheim California…right by Disneyland!

My ailment started with my sinuses and worked its way to my lungs. My lung involvement is what prompted my doctors to question WG.

I currently receive treatment in Irvine, California through Kaiser Permanente.

As far as I can tell, my journey started towards the end of September 2010.   My husband, Jeff and I had just been married in March 2010, and were enjoying our new life together and looking forward to the future. I love being outdoors, and have always been pretty active…I love to walk, run, camp, hike, go to the beach…a typical Southern California girl.

It all started innocently enough, I started getting a runny nose and feeling a little “under the weather”. I went to the doctor and was sent home with seasonal allergies and some OTC medications.

The first weekend of October, I attempted my first 10K race. I made it out about 2 miles, and I had to turn back and abandon the race all together. I just couldn’t find the energy to complete it and felt awful. At first, I attributed it to “biting off more than I could chew” in a race. I usually run flat 5K courses, this was twice my regular distance and had multiple elevation changes, so I said to myself “lesson learned, stick to what you know” and didn’t think much of it at first.

By the time I got home from my attempted race, I was running a pretty high fever and now I felt sick. I went straight to bed and slept until my fever broke. For the next few days, I was doing all the regular at home cold remedies…chicken soup, saltine crackers, Gatorade, Sprite, Nyquil…all the things that usually helped me get back on my feet again. When I wasn’t feeling any better by the end of the week, I went to the doctor again. I was told that the colds and flues going around this year were just really strong, get more rest and I should be better in about a week. Basically, just keep doing what I’ve been doing. So I did.

Two weeks later, I was still feeling horrible. I didn’t think this was normal for someone my age to have a cold for this long. At this time, I had been coughing quite a bit, and one morning I started coughing up blood. This was truly alarming, so I made yet another doctor’s appointment for that afternoon. The doctor I saw said that my constant coughing for weeks had caused a slight case of bronchitis and a few specks of blood was nothing to be alarmed about. I insisted that it was more than a few specks, that I was coughing up clots of blood, and she still shrugged it off like I was being a hypochondriac. Not wanting to hear that it was something more serious, I took her word for it, and told myself I was making more of it than it really was. I was given some OTC cold medicine along with a nasal spray to help with my “seasonal allergies”.

By the time Thanksgiving came around, I was still sick. The Monday before Thanksgiving, I made yet another appointment to see another doctor because something was clearly not right…no healthy 26 year old should have a cold for 3 months with no end in sight. I took my lunch break to go to my doctor’s appointment with Dr. N, and told her all my symptoms and what had brought me to her thus far. I had general cold symptoms including fevers, runny nose, congestion, coughing and sometimes coughing up blood, fatigue and just plain feeling tired all the time, and I had started getting a pain in my upper right back that got worse with deep breaths. She sent me downstairs to have a chest x-ray.

When I got back to the exam room, Dr.N pulled up my x-ray on the screen and showed me some cloudiness in my lung where I had complained of back pain. After consulting with another doctor, she diagnosed me with pneumonia. SCARY!   But I was glad that someone had finally listened to me and I could start feeling better. I was sent home with antibiotics and an inhaler, and told to come back in two weeks for a follow up chest x-ray to make sure everything was clearing up. I took the next week off of work per Dr.N’s orders, rested, took in lots of fluids…basically I did everything I was told to do.

Two weeks later, I went in to have my follow up chest x-ray on Monday. I was still not feeling great, but with all the rest I had, I was feeling marginally better. By Wednesday, I hadn’t received a call to follow up on the x-ray, so I emailed my personal physician, Dr. D, to make a follow up appointment. If anything, I just wanted to hear that I was getting better. I went in that morning, and Dr.D came in looking somewhat confused. She asked why I had made the last minute appointment to see her; I looked like I was doing fine. I explained to her everything that had been going on for the past couple months, and she excused herself for a moment to review my x-rays and the other doctors notes in my file. Since my symptoms had started in September, I had seen 4 different doctors. She came back a couple minutes later, now looking concerned. I asked if my x-rays looked better, and she said “no”. She had a call out for a pulmonary specialist to take a look at them because they had not improved from the first set taken before Thanksgiving. She then went on to tell me that she was calling over to the hospital to find a room for me, she was admitting me into the hospital until they could figure out what was wrong with me.

To me, this could not have come at a worse time. My best friend from High School was getting married the following weekend, and later tonight was her rehearsal dinner. I was a bridesmaid in her wedding and HAD to be there! I begged Dr.D not to put me into the hospital right away, but she apologized and said that she had no choice. Her first instinct was that I had Tuberculosis, and being highly contagious, she couldn’t in good conscience let me out into the general public and risk spreading it. So I called my Mom and Jeff to come pick me and my car up from Dr.D’s office and drive me over to the hospital.

Now Wednesday, December 8, 2010, I was in the hospital not really sure what was wrong with me. I honestly thought that everyone was overreacting a bit, I felt fine…or maybe it was just a version of “fine” that I had become accustomed to in the past few months. Because they were looking at TB as a possibility, I was put in an isolation room, only Jeff and my parents were allowed in to see me.

Over the next day or two, a pulmonary specialist, an infectious disease specialist, and the doctor overseeing the hospital floor came in to talk with me. They all showed me my most recent x-ray, and pointed out three spots in my right lung that they called “cavitary lesions”. They went on to explain that this could be a number of different things; Tuberculosis or a fungus/ spore growing in my lung that could both be cleared up with an antibiotic, or something more serious like lung cancer…luckily I didn’t hear this part when I was in the hospital, my mom told me about it later. With so much information being thrown at you, it’s easy to miss parts here and there. Thankfully, I had Jeff and my Mom there to listen when I couldn’t and fill in the blanks.

By Friday, they had been running all sorts of blood tests, and I had been giving sputum samples to be tested for TB. They were also going to send me in for a CT guided lung biopsy to try and figure out what exactly those lesions were. When my pulmonary specialist, Dr.C, came in to see me Friday afternoon, he was giving me a rundown of what they knew so far, and said hopefully when he came back Monday, they would have answers for me…this meant they expected me to be in the hospital ALL WEEKEND! I again became overwhelmingly upset and tried through my tears to explain to Dr.C that my best friend’s wedding was Sunday, and I couldn’t miss this important event in her life, she was counting on me. Through my hysterics, my nose started bleeding profusely. Dr. C was shocked. He asked me why I had not listed this as a symptom before. I told him I had only had one other bloody nose in the past couple months, but everyone gets bloody noses, especially in the dry weather this time of year, and I thought nothing of it. He said he’d be right back, and came back about 5 minutes later with test results and a diagnosis.

He told me “you have Wegener’s Granulomatosis”, and briefly explained that it was an auto-immune disease that would explain my sinus symptoms and caviary lesions in my lung, and the positive c-ANCA blood test he held in his hand. He said that the lung biopsy would confirm his diagnosis, but he was 90% certain that this is what I had.

Thinking that I was in the hospital for no real reason, this was shocking news, and I still didn’t really believe it. The infectious disease doctor came back in Friday evening before I was scheduled for my biopsy, and he assured me that he thought I had a fungus, not Wegener’s. I was a bit relieved to hear that, but still petrified that he was wrong.

A Rheumatologist, Dr. M, was contacted and came in to see me on Saturday morning. She went over my symptoms and gave me a quick check up, and started explaining my treatment options to me. At this point, my head was still reeling from all that had transpired in the last 24 hours. I told Dr.M that I wasn’t ready to hear anything until my lung biopsy came back with a diagnosis. She was reluctant, but understood and said we could talk about it in a week when my results came back.

After all this, I had finally been cleared of TB, taken out of isolation and was being prepped for discharge from the hospital. Around 4:00pm on Saturday, I finally got in the car to go home. I was barely able to meet my friend and the other bridesmaids that night to make our bouquets, but I made the wedding the next day, although weak and not myself, I was so glad to be there and not miss this milestone in her life…she had been in my wedding only months before, I wanted to return the favor as best I could.

The next two weeks were the hardest I have ever gone though. I hadn’t read anything on Wegener’s up to the point of my friend’s wedding, so after Jeff and I got home, I sat down and looked it up…this was a mistake in hindsight. The first thing I read was from Wikipedia, this is what I saw:
Wegener's Granulomatosis is a form of vasculitis (inflammation of blood vessels) that affects the lungs, kidneys and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression.^[1] Five-year survival is up to 87%, with some of the mortality due to toxicity of treatment. It is named after Dr. Friedrich Wegener, who described the disease in 1936.^[2]

When I read this, I saw “Five year survival” and thought I had up to 5 years to live. This was devastating! I’m 26 years old….Jeff and I haven’t even been married for a year….we wanted to start a family…..how could this happen to us! We both sat down and cried for what seemed like hours. Now it was a waiting game. My biopsy wouldn’t be back for another week, and I just had to pray that they were wrong about my initial diagnosis….I just couldn’t believe this was happening.

That following Thursday, I went in to see Dr.C for the first time after getting out of the hospital, and he gave me the results of my biopsy…inconclusive. This was a big letdown. We were expecting to make big decisions based on the results of this test. He said, regardless of the biopsy, he was still diagnosing me with Wegener’s based on all my symptoms and positive c-ANCA test. At this point, I had to accept it. I had spent the week hoping for a miracle, but there was no denying it at this point….I have Wegener’s Granulomatosis….and in a weird way, it was comforting to hear a diagnosis, even though it was hard to hear, at least now I can start treatment and recovery.

My mom and Jeff were at this appointment with me. I think they both needed to hear the doctor say the words as much as I did. Jeff and I had another bout of crying and getting all our emotions out when we got into the car, and I met my mom at home and cried with her, and later that day when my dad got home, we cried again. As upset as I was about everything, what upset me the most in the beginning was the guilt I felt for putting my family through all of this. I am an extremely self sufficient person, and I hated being a burden on them, not only because I wasn’t strong enough to do anything for myself physically, but I felt like an emotional burden as well. It took me weeks to accept that this happened to me and there is nothing I could do to change it. After leaving Dr.C’s office, I started on 60mg of Prednisone daily per his orders, until I discussed my other options with my Rheumatologist.

At the advice of my doctors, before deciding on what treatment I would go though, Jeff and I made an appointment to meet with a fertility specialist, Dr. T. This was the HARDEST part of my diagnosis for me.   We are newlyweds, and we talked about starting a family almost every day…2011 was going to be all about babies for us. So, on Monday morning, we drove down to the Dr. T’s office. He explained that given the toxicity of my future treatment, the most viable option for us would be to preserve embryos. However, this process would take about 6 weeks, and for those of you who know anything about how fast WG can take a turn for the worst, 6 weeks is too long without any treatment. It was also not covered by our insurance and was extremely costly. To add insult to injury, the most common treatment, Cyclophosphamide, would most likely put me into an early menopause from which I have a 50/50 chance of recovering from…so even if we spent the thousands of dollars and I went through the highly invasive procedure of preserving my eggs, we only had a 50/50 shot that I may ever be able to carry them. On top of that, having Wegener’s already puts me in the “high risk pregnancy” category, and I may never be able to carry a baby full term, my body just may not be strong enough to handle it.

After leaving the fertility doctor very sad and disheartened, Jeff and I met up with my Mom, and we headed down to my first official appointment with my Rheumatologist. We told Dr. M what had been explained to us, and asked about some other options we read about online. Rixuximab is a newer medication used in Rheumatiod Arthritis that doesn’t affect fertility, but there is only about 5 years of research with WG patients done to back it up. My husband, being the wonderful man he is, told me that it is my body and he would support any decision I made regarding my treatment. We talked about it, and decided to go with the tried and true method of Cyclophosphamide as my main immunosuppressant.

As of January 3^rd, I stared my Cyclophosphamide at 100mg per day, and two weeks later it was upped to the full dose of 150mg per day. I take this concurrently with Bactrim and Prednisone.

Within a week or two, I started feeling exponentially better. My sinus’ were not as inflamed, my breathing was less labored, and I started to get a little bit of energy back, not quite back to my old self, but I am now able to do some chores around the house, and get through the work day without a nap, and every week seems better than the week before, but hopefully not as good as tomorrow.

It took about 2-4 weeks for me to start feeling the side effects of my medications. The Prednisone had caused me to gain weight, and my face looked “swollen”, for lack of a better word. I started getting acne and mood swings like I was a teenager again. Some other common side effects to Prednisone are insomnia and acid reflux; I have been fortunate to only experience these minimally. The Cyclophosphamide made me more tired than usual, and I get upset stomachs and nausea. I go in for weekly blood tests to monitor my disease activity and how my body is responding to the medications.

All in all, I think I have tolerated the drugs pretty well. In my first week of treatment, I had one bad reaction with my Bactrim and Cyclophosphamide, I woke up with sweats and excruciating joint pain in my legs, to the point I could barely explain it to the nurse on the phone without hyperventilating. It subsided about an hour after I took my Prednisone, and when I lowered my Bactrim dose the following week, I didn’t experience the issue again, thank goodness.

Now March 2011, I am still in the midst of my initial treatment, but things have already started to improve. In mid January, I had another chest x-ray done, and the nodules in my right lung had already began to heal. My Rheumatologist has also been slowly weaning me off of Prednisone as my symptoms decrease week by week.

In the beginning, I had mostly bad days, both physically and emotionally, to the point that getting out of bed seemed like a waste of my time…what was the point. As I started to feel a little better and read more about Wegener’s, I realized that there was still so much in my life to be thankful for. I realized that people do recover from this disease, and thrive, and there was no reason why I couldn’t be one of them. I started looking forward to things again and focusing on the positive things around me. My husband and family helped a lot with this, but a huge help was The Wegener’s Granulomatosis Disease Support Forum.

To someone who has never been diagnosed with a rare, potentially life threatening illness, it is hard to describe the sense of helplessness and isolation you feel. As much as my family was there to support me, I still felt alone, like no one understood what I was going through. When Jeff showed me this site, at first, I was hesitant to check it out, but after a few weeks, I started reading daily, as I still do. To have someone to talk to who knows what I’m going through on a personal level is incredibly helpful, their support and advice has been truly invaluable in my recovery process.

My advice to anyone who is being diagnosed with Wegener’s, or thinks they may have WG…trust your instincts. You know your body, and if you think something is wrong, speak up. Don’t let someone tell you it’s nothing, if you feel in your heart that something isn’t right. I would also advise anyone who thinks they are having symptoms of Wegener’s keep a small journal and write down how you are feeling on a daily basis. This way, when you do meet with a medical professional, you can accurately explain to them everything you have experienced and get the proper treatment as soon as possible.

For anyone who has been recently diagnosed…chin up. It is hard in the beginning, and you will have bad days, but you will also have good days, and you will get better. Take your bad days in stride. And when you’re feeling good, do something you enjoy to take your mind off all the craziness that WG brings. After being diagnosed, I had a hard time getting back to everyday activities; coming home for the first time, going back to work; I looked around and saw pictures of myself from when I was “healthy” and carefree, and it made me really sad. But, day by day, I realized how important a sense of normalcy can be. Getting back to your regular life prior to diagnosis is a huge stepping stone that no one tells you about, but it is so important. Lastly, talking about my disease and experiences helped me cope with having Wegener’s. Every time I explained Wegener’s to someone new, I understood it a little better myself, and sometimes I was asked questions that prompted me to do more research. With every person I told, it became easier to talk about, and a little easier to accept.

Stay positive and take it one day at a time.

In closing, I would like to thank my husband, Jeff, for being SO supportive. I think the next book should be about being married to a Weggie…it is a rollercoaster of emotions for our spouses and loved ones too, but they don’t have any medication to help them through it…I’m kidding of course, but they really deserve credit for putting up with all the changes and side effects that Wegner’s brings, and loving us in spite of it. I would also like to thank my parents; my Mom for always being that voice in my head that makes me question everything…it led me to diagnosis. And my dad for being a positive example of strength and recovery in my life. And lastly, I want to thank Dr. Chan. He has been on top of my case since day one, and has always been honest with me, even when I didn’t want to hear it, and I can’t express enough how grateful I am for that.

In the next story you'll read about Bob...

My name is Bob, and I’m a Weggie. I was born in Bluffton, Ohio in the Spring of 1972. Growing up my family moved around a lot as my dad joined the Air Force when I was in third grade. There were a lot of challenges that came with this, I’m a reasonably shy person so adjusting with each move could be a bit of a challenge. Overall though I wouldn’t trade that time for anything as I saw so many fantastic things over the years.

I spent most of my school years dreaming of growing up and becoming a pilot someday, but in my senior year of high school my eyes decided it was time to go nearsighted and my plans were changed. Not having a clear plan at this point I joined the Air Force myself. I spent a few years in the service including time spend in Saudi Arabia during Operation Desert Storm. After a few years in the service I discovered I loved working with computers and I got out to attend college.

During college I met my wife, who was a friend of my younger sister. We’ve been married now going on 16 years. After some difficulty in finding a job after college I eventually got into computer programming and have been working as a programmer/developer in the 15 years since.

The downside of the computer based job is that I became very sedentary in my lifestyle. Over 14 years I gained a bunch of weight and eventually started causing myself health related issues. During a surgery for a perforated Gall Bladder, a final problem arose when I discovered I’d developed problems with my liver based on my weight. I made the relatively serious decision to get a bariatric surgery before I did irreparable damage to my liver.

I won’t pretend that all these issues, and the eventual Wegener’s diagnosis were not difficult to deal with. I had my moments of anger and denial of course. But I believe that God brings all things into your life to continually conform his followers into the likeness of Christ. Once I was able to calm down from the initial diagnosis I was able to take confidence that all things he brings into my life are to transform me.

For me the Wegener’s ride started innocently enough. In March 2010 I returned from a Caribbean Cruise. Shortly after returning I developed a chest infection. Over the next two months I was treated with antibiotics twice. Eventually they decided I had asthma and I was treated with a Prednisone dose pack. It was right after this that things really deteriorated.

In early May I took part in the Susan J. Komen race for the cure in Columbus with many of my co-workers. I had been working as much as I could to get physically active and was in the best shape in years. The chest “cold” had made it difficult but I was still hitting the gym as much as possible.

The race made for a great day, but the next day I felt like someone had beat on my legs with a baseball bat. I thought I had pushed a bit too hard on the race perhaps and expected that I’d see some relief shortly. Instead I got a deluge of new and very odd symptoms. The pain was persistent, but tended to move around the body, though the worse I got the more areas were hurting. Eventually, I had pain in my ankles, knees, wrists, index fingers, horrible pain on the outside of my hands, and finally in the shoulders and neck.

Next I developed dizziness and headaches nearly every day. The dizziness scared me the most as I found that I was having issues where I was very close to passing out fairly regularly. The headaches were most like sinus headaches but were of an odd slightly mild intensity, but way more persistent.

It wasn’t until about 3 weeks into the mess that I started having massive night sweats. At night I’d wake to find I was running a fever and drenched in sweat. During the month of May I lost a total of 30 pounds. In the course of 6 months I’d gone from obese to way too thin to most people I was talking with.

I tried to go to the Emergency room in the middle of May. At this visit I was treated horribly at what is supposed to be one of the finest Hospitals in the country. They clearly thought that I was there seeking pain meds and wrote me a script for Percocet. Now I’ve been through some serious surgeries in the last couple of years and quite honestly I don’t do pain medication unless I’m in horrid shape. I was very angry, and refused to fill this script.

Out of this visit they requested I see a Rheumatologist, even though there was supposedly nothing wrong on my bloodwork. They did follow up though stating that I had a possible positive for Malaria, which oddly fit the symptoms to an extent. So I had a follow up scheduled with infectious disease as well. Of course I had to wait several weeks for both followups… One odd thing that nobody noted except my mother (who is a nurse) is that my sedimentation rate (ESR) and C-Reactive Protein (CRP) were off the chart.

The first follow up was with the Infectious Disease doctor. I went to a competing hospital due to the bad taste in my mouth from the Emergency room. After a multitude of tests they said there was no way I had Malaria. At this point it was extremely disappointing as I had hoped for any sort of diagnosis.

Disappointed I went to the Rheumatologist, again back with the first hospital. She basically wrote everything off saying I was negative for RA and Lupus and she was sure it was some sort of infectious disease.

At this point I was truly desperate, it was getting so painful to walk that I had to sit on my butt and slide down the stairs in the morning to get down. At this point I couldn’t handle the continual passing of the buck and went to another emergency room. The ER doctor at the competing Hospital treated me like I shouldn’t be there, but admitted me to the Hospital at my insistence.

I spent three days in the Hospital while they tried to figure anything out. I think the Infectious Disease stuff had their attention so much they couldn’t figure anything out. I was desperate at this point, we were joked a lot that we needed our version of House to figure out what was going on. At this point my blood pressure was constantly low, my hemoglobin had started dropping alarmingly, and CRP and ESR had actually capped out. But they couldn’t figure anything out. At this point they made me an appointment with a Hematologist and ENT doctor, separating my symptoms into what were surely two different issues.

My “House” came in the form of Doctor Nse Ntukidem, my Hematologist. After reviewing my lab work and examining my now seriously swollen knees he said he thought I definitely had something Rheumatologic going on. His diagnosis was not the correct one, but he got me on the right track finally and got me an appointment with the first Rheumatologist that finally did broader bloodwork on me. In my testing to rule out cancer they noted that I had some thickening of the trachea and I was asked to confer with the ENT doctor on this.

In the interim I also saw a ENT doctor, who thought the thickening they saw on my trachea was caused by acid reflux. He gave me a script for Nexium and I was on my way. I thought I was at least going to get some relief from part of my symptoms.

At this point I was sure that the pain in the knees was such I couldn’t handle no treatment until the Rheumy appointment. I got with my family doctor and informed him of what the hematologist suspected. The normal treatment was prednisone. He prescribed pred for me and after getting approval to start by my Hematologist I started, to almost immediate relief. Blood work improved, pain subsided, swelling dropped. The large doses of prednisone were helping a ton.

At the Rheumatologist my swollen joints were examined. His first suspicion was a rheumatologic infection of some sort, an important note here is that he was missing part of my symptoms as I had attributed them to “acid reflux”. He was concerned at the size of the prednisone dose and started me on a taper. The blood work he ordered came back with still elevated CRP and ESR, and one new note. My C-ANCA was elevated. I took this paper home with me and on looking it up was faced with Wegener’s Granulomatosis.

After reading the symptoms I found a near perfect match. I was also terrified seeing a lovely comment regarding fatality rate, what the heck did I have??? I called the Rheumatologist back for a quicker follow up. I also started looking around and found that the Vasculits Foundation suggested the Cleveland clinic, which is just a few hours up the interstate from me.

The Rheumatologist almost immediately came around to thinking it was Wegener’s when I revealed my missing symptoms and the trachea issues I had previously omitted. He was very supportive of me following up with the specialists at the Cleveland Clinic.

This was an odd time for me. There was relief and being pretty sure I’d found my problem, and concern that it was something that had the distinct possibility of being fatal. I found a great forum online at Wegeners-Granulomatosis.com where people were supportive and informative. I learned a lot about something pretty obscure fairly quickly, I also learned that my instinct to go to the Cleveland Clinic was the right one. With this disease proper treatment is extremely important.

My Doctor at the Clinic is Doctor Villa Forte. She has been great at reassuring me and is very knowledgeable about what is a very obscure disease. I decided that continuing with treatment through the clinic was the way to go. I could have returned to my rheumatologist, but a Doctor who had dealt with hundreds of Wegner’s patients was surely the best choice.

I was finally assured that I do indeed have Wegener’s Granulomatosis, and that it was causing Tracheal Stenosis. This is a gradual closing of the trachea below the voice box. It was the cause of my horrible coughing I’d been experiencing for months, and the shortness of breath I was now experiencing with aerobic activity.

Treatment was to involve cyclophosphomide or Cytoxan taken daily. At this point I began to understand just how bad prednisone was for me, and was disappointed that I kept having to slow my taper as I was constantly on the edge of pain.

Cytoxan is a chemotherapy drug, and has many bad side effects including bladder cancer if you do not properly evacuate the bladder regularly. For me the biggest side effect I noted was serious fatigue that hit about 4 hours after taking it. I’ve never been a napper, but found the need for a nap in the afternoons while I was under this treatment.

I am quite thankful that my employer, DSW Shoe Warehouse, was willing to be flexible for me and allow me to work part of my day from home. This allowed me to stay as an active employee during the treatment. Towards the end of my treatment on Cytoxan I found that I started to suffer from less fatigue, but as soon as I was off the drug I found that I was suddenly far less fatigued and able to work full days at work again.

I am currently taking Methotrexate as a follow up to the Cytoxan. Methotrexate is also a chemotherapy drug, but is considerably easier on the body. My previous liver issues make it very important that I continue to get my monthly lab work to keep track of liver enzymes and make sure they stay within a healthy range.

So far I have been lucky enough to avoid a flare. I’m told that if I am careful to stick with the treatment and lab work the likelihood of a flare is greatly reduced. I hope this is true as I really have no desire to return to the full Wegener’s experience again. J

At this point I find my life is slowly returning to normal, or my “new normal” as most Weggies seem to deem it. I’m certainly not anywhere close to where I was last March, but I do feel considerably better than any time since May.

Physically, the biggest change I’ve experienced is that of being weaker than I was before and having to tone back a lot of activity. I hope that with remission I might be able to return to a lot of my previous physical activities. I have slowly been able to start doing some exercising again. I have to take it carefully though as the prednisone makes it such that it is easy to injure oneself.

Mentally or emotionally, I feel like this has given me a new perspective on the suffering of others. Until you are in this situation you don’t really understand chronic pain at all. Abstractly, I wasn’t as understanding of issues with medical care that others received as well. From inability to get health coverage, to shoddy care from practitioners I feel like I have a deeper empathy for others now.

I’d like to thank my wife, Lucinda, for putting up with me through all of this, prednisone is a harsh mistress at times J. Thanks to my kids for reminding me what I have to work for. Thanks to my parents and sister for caring and pushing for a diagnosis in all of this.

Here's Moshe's story...

I was diagnosed in 2006 at the age of 58.

I was born in Argentina and when I was 13 years old my family moved to Israel. I have two lovely granddaughters and lives in a small village. We liked to travel in the country side by foot and I was learning every morning Talmud before work which is a very brain developer.

I worked as Safety Work Coordinator in several industries like mining, laboratories, food, plastic, construction, so my work was very interesting and challenging.

At first I had symptoms of ache in my right ear and it developed in few days to swelling of my face and s a result the jaw moved aside causing severe pain and limited jaw open so I was able to eat only liquid food. In that situation I could sleep only by lying in the left side without pain.
At first I wasn’t diagnosed, the doctors thought I had an ear or saliva gland infection and was treated by pain relief and antibiotics for about 4 month in that period I was hospitalized in department of Otolaryngology and at home at intervals of weeks and also tried to work between

In my work I had to fly in the morning and at the end of the day fly back home. One day on my way home, my ear begin to discharge and once again I was hospitalized. They made several tests like MRI, biopsy of the saliva gland , bacterial analyses, still they did not diagnose the cause of my health problem

Because I had no relief of my symptoms I tried acupuncture. When I got to him, he made me a diagnosis by looking at my tongue and said that I have also a problem in my kidney function, but he will treat first the saliva gland.
After four sessions there was no more swelling and the jaw returned to its position but then I started to have new symptoms: my feet where like a balloons, my heart bit was very fast and I was short of breathing. I had severe pain in my back that forced me to sleep while sitting that night.

In the next morning I went to the emergency and was diagnosed as severe renal failure.
The first one who suggested I might have W.G was a doctor who worked in the emergency. He made some calls to several experts: heart, nephrologists, internal who made all the tests and once again subscribe me an antibiotics whom I refused to take.

Meanwhile they kept doing more tests, one is Anca which came positive and I was finely diagnosed as a W.G . the head of the internal department came and explain to me shortly about the disease and the treatment for it and the risks.
As a complication of the disease I hade renal failure and bleeding internal in my lungs so I was admitted to the intensive care unit for hemodialysis I also developed DVT in both legs.

In intensive care they tried to put me on hemodialysis but the pipes where clogged by blood coagulation and it was impossible to use heparin because of my lung bleeding. Then I was put on Citrate Dialysis for 8 hours every session in intervals of one day and also had 5 sessions of Plasma Phorezis I was treated with Cytoxan and Prednisone before I was released from intensive care they tried to put a line in the artery in the legs but it became clogged every time so a last they insert me the line in a venous in my neck to prevent coagulation

I was released from Intensive Care after 10 days and they prepared me to peritoneal dialysis

I perform hemodialysis at the end of October, In November 2006 I stared the treatment of PD till the beginning of January my createnine dropped down to 2.8 and the nephrologists instruct me to do PD twice a day till the createnine will go down to 2.5 and then stopped the treatment but I was with the line at June 2007 for precaution

My drugs included : Cytoxan and Prednisone for W.G.,
As a result of my renal failure I had disfunction of the adrenal gland and my bone morrow didn't producing red cells, I developed high blood pressure so I took Enalapril for the blood pressure and folic acid and B12, B6 and Aranesp (a hormone like that helps to create new cells).
Resprim took care of my immune depressed system as a side affect of the prednisone.

In March 2007 probably as a result of my immune depressed, I got shingles.
It hurts my head and the left side of my face . I had an edema of my feet and lower quadrant and my face was so swelling I couldn't see. Again I was hospitalized and got treatment for shingles by Zovirax
Shingles hurts my sensor nerves. the neurogenic pain of the singles begin after I was released from hospital. This pain interfere the most to my well being. The pain shoots abruptness like a white-hot iron cutting throe my skin. I found myself screaming, iriitating . I couldn’t sleep properly couldn't go anywhere, couldn’t drive - couldn’t do anything – it shut me down .
They tried to treat my neurogenic pain by Elatrolet with no success.

Till today I cannot sleep on the right side I must lay on the left side with a towel under my head .
Also it is not pleasant in a windy weather or to drive with an open window.

My life changed. I could no work in my last position because it was involved with long driving and field work with long walks.

I have an extensive knowledge so I used to maintain my house by myself even repairing my own car and my friends car. Now I cant do any of those I get too tired after a small strain.

Now that my Wegener is in control, I am trying to do what ever I can. I help my daughter in law and my sun with my two granddaughters and do some little repairs in the house and in my yard. I try to make the most of my day under my limitation, I read and study

From my experience I have some advice

1.      Never take your doctors for granted , ask them questions without fear.

2.      get information from books and web about your treatment and drugs , side effects, and interaction between drugs and discuss it with your doctors.

3.      always ask for second opinion and check every test the doctors suggest you if it is really needed.

I must thank my great family for being with me in the hard days and specially to my wife for her support and strength and being at my side in every meaning of that.

I am very lucky to be followed up in nephrology all the staff, the nurses, officials and especially my doctor who is not only an excellent doctor he is a humanitarian.

Here's Geoff's story....

Glad to be Diagnosed

It’s a Fact of Life, we all fall ill at some time or other, irrespective of age or other personal circumstances. Some of us endeavour to ‘Keep Fit’ and compete with ‘Old Father Time’ as our bodies come under increasing pressure of living in today’s madcap world. When we do fall ill we either self diagnose and take a familiar effective remedy, or call on our local doctor to sort us out with perhaps a stronger medicine. What we do struggle to come to terms with, is when we find ourselves feeling gradually worse, day by day and yet unable to find a remedy or even cause. Such was the situation I found myself in at Christmas Time 2007.

Syptoms:

What began feeling like flu, developed into a variety of symptoms that confused me. Initially, I felt shivery and cold; I would huddle over the fire trying to get warm. I experienced headaches and painful joints throughout my body, especially my arms and across my shoulders/neck. I suffered with night sweats; swollen glands in my neck which restricted my ability to turn my head and even eat! Next followed an infection of the inner ear which caused vertigo and nausea; coupled with an infection within my right nostril. This was followed by two large lesions on the side of my tongue which were quite painful as they rubbed against my teeth.

I was also aware of the discomfort caused by compression of my skin, say with socks and shoes. Laying in bed the back of my heels would feel very sore where they had been ‘pressing’ down against the bed. All of these symptoms were cast against the backdrop of ever increasing fatigue within my whole body. I felt as if I was gliding slowly down into a black abyss. Matters came to a head when I developed a chest infection and started to cough up blood. A blood test showed that I was anaemic and an X-Ray highlighted problems with my lungs.

Fortunately additional blood tests were analysed and a preliminary diagnosis of Wegeners saw me admitted to hospital. In all just under 4 months from my first feeling unwell.

The Story:

I was 56 years old when all this started and had been very fortunate to enjoy good health all my life. I had been a Land Surveyor since I left school and enjoyed working outdoors, in all sorts of weather, well I do live in England! My job gave me a certain level of fitness and I belonged to a local gym which I attended 3 or 4 times a week. A 20km bike ride against the clock would be my normal ‘warm up’ before I moved onto the various machines of torture! Looking back I feel that my recovery was helped considerably by my good physical condition. The doctors in the hospital were amazed at the rapid improvement in my lungs after just 4 days of treatment and this gave me a positive uplift.

It may be strange to the reader but one of my over riding feelings at the time was that I was happy to have been finally diagnosed. Yes the initial words of the administrator who said” We believe you have Wegeners Granulomatosis and I have to tell you that it’s fatal” were rather bizarrely, music to my ears. The pain and fatigue that had slowly but surely taken a firm hold on me, coupled with the fear of the unknown and frustration of numerous trips to see various doctors and consultants had left me at the lowest point in my life.

Hindsight is a wonderful thing, and of course we can now look back at the various incidents when perhaps a diagnosis could have been made earlier. I have no recriminations, as, compared to many others who suffer with this disease, I am very fortunate, but I do spare a thought for those who have struggled on for a long time before the correct treatment has finally been administered. I can laugh now when I think of the time I had my ‘varicose veins’ treated in my legs or when I had a septoplasty because of a deviated septum. Being given Pain killers and told to go away and let the virus run its course, now seems a ludicrous option. So, you can appreciate, why when I finally had my diagnosis, I felt relieved.

Being told that you have to go immediately into hospital is of course a worrying aspect to take on board. I recall vividly that first sleepless night, being moved from one bed to another as the administration turned into a watching brief. The patient in the next bed was woken every couple of hours so that various tests and samples could be taken. The strange noises that pervade a hospital and the lighting only added to the sleeplessness. The following morning I was seen by a doctor and things happened quickly. I was told that a hospital in Cambridge, called Addenbrookes, had a dedicated Vasculitus department that had experience in treating my condition. Little did I realise just how lucky I was that day.

They arranged for me to be transferred in an ambulance, and I was wheeled out on a stretcher just as my wife and daughter appeared to hand me a clean set of pyjamas, fresh from a large department store in the local town, well one must try to keep up appearances! The ambulance set off with me laid out on the stretcher which was bolted down in the back. We hadn’t gone too far when I started to feel very sick ( I could blame it on the inner ear infection but if truth be known I had always been a ‘bad’ passenger) I was given a sick bucket which I effortlessly filled and I asked if I could sit up on the stretcher. This only allowed me to go another couple of miles before I urgently required the use of the bucket again and that’s how I ended up in the front seat next to the driver, suitably clad in my dressing gown, as we tore up towards Cambridge. I was dying to reach up and switch on the siren and flashing lights but it would have been an abuse of their kindness to me! I just needed to look ahead and see the horizon I guess.

The Hospital was massive but welcoming. The tests endless as they checked out my lung capacity and function; a CT scan and more blood tests followed and then I had my first visit from Dr Jayne, who explained to me exactly what my condition meant. I had 5 treatments of Plasma Exchange which I likened to having all the ‘bad boys’ taken out of my blood. The other analogy I had was that they were Pac-men, given to racing round my body, munching on all the tasty bits!! Sometimes my imagination can run away with me. Being so anaemic, I was given 3 blood transfusions towards the end of my stay. In all I was there for 2 weeks during which I ‘celebrated’ my birthday. Being woken by 3 nurses pulling back the screens around my bed at 6.30 singing a chorus or two of ‘Happy Birthday to You’ will live with me for ever. As will the hand made cards which they had ‘coloured in’ during their night shift.

The whole experience now seems to have a certain dream like quality. I was in a special ward, and compared to the other patients, I seemed the least ill. I could get out of bed and ‘pop down’ to the shops on the main concourse and pick up the daily papers for the other guys on my ward. It might take me some time, as I struggled to catch my breath, but I was determined to maintain some sense of normality to my life. People with Wegeners don’t appear to be ill compared with other patients, and as I looked at the people waiting for transplants etc I told myself that I had had a lucky escape. As my Mum would always tell me “There’s always someone worse off than you”, how right she was.

It was only when I went home that things started to sink in. I struggled to climb the stairs to go to bed and found it impossible to even drive the car a mile. I had to convalesce and was told about 6 months. Due to several reasons, this proved to be a non starter (Money makes the world go around!) and I found myself back at work after 7 weeks. Luckily my employer adopted a very supportive stance, and I worked half days to start with until I got my strength back. Maybe it was the high steroid dose but I felt fit to bust and seemed to have bags of energy. I was determined to show that my life was back on course. I set myself personal goals and the rest of the year seemed to be a succession of tick the boxes as I attempted to prove to myself and my friends that all was well.

After my first ‘anniversary’ life changed. Remembering to take all the pills was not so much a novelty. My illness was no longer the centre of conversation and I had to adapt to ‘real normality!’ I was still attending outpatients on a very regular basis, starting weekly and then finally going out to every 2 months. After 18 months, things took a turn for the worse, but as in keeping with the illness, it was a slow gradual glide into the black abyss again. You really have to listen to your body and be aware of all of the subtleties associated with this illness. I ended up at the end of the year having my first ‘major flare’. I was still able to go to work but really struggled to keep alert. Most evenings I would slope off to bed really early and for most of the weekend I would have to ‘lay up’ as my energy levels would be zero. The situation was addressed by a course of cyclosulphamide administered in the outpatients at Addenbrookes. I would feel sick and nauseous after the treatment and struggled to keep going. Often I would have to give in and take to my bed. I can freely admit now, it is my favourite place!! Looking back I guess its easy to say that I tried to carry on normally and ignored the fact that I was suffering from a life changing illness. I have learned that whatever you try to do, its only permissible when the Wegs Dog allows you, it is the master and we have to listen when it barks, however some times we can slip a muzzle on it and for a short time pretend we have it under control.

I have continued to work full time and this year sees my one and only daughter get married. My target has become very focused and that is to attain a reasonable degree of fitness and to try an lose a few pounds of excess weight. I have put on 30 lbs during the steroid treatment and call it vanity, but I want to be able to look at the wedding pictures and concentrate on my beautiful daughter and not flinch at the ‘blob’ walking her down the aisle.

I still haven’t lost my relief at being diagnosed because when alls said and done, it has brought about an acute awareness in my life. Just exactly what the true worth of ‘things’ are in our material world, and what I need to do to ensure that the precious time that I have been given is not wasted.

and now for Mike's story....

Late August 2011, I started coughing up blood; I started having fevers, low grade but continuously thru-out the day and night. I started with severe sweats and eventually noticed the sweats where tied in with my fevers, I was so tired, I would work and it would take me 3x as long to complete my work.

I currently live in Chatham Ontario, Canada and receive treatment in London Ontario

The summer of 2011 was extremely hot here in Chatham, unusually so. I am self –employed and have been for about 30 years installing floor coverings. My hobby is wood-working. I craft wooden cremation urns, I would build the urns and my wife would finish them. This is to be our retirement plan. I was so tired thru-out the entire summer, I would go to work, come home just exhausted. I really thought little of this, just kept blaming it on the heat. We would usually spend a lot of our time at our friends on the lake near-by but I really didn’t have the energy. I just waited for the heat wave to pass. By the end of August I had a bit of a cough, I would struggle to get the phlegm out. A couple of times I noticed that it was what I called a “brownish color”. I had mentioned it to my wife but I think the fact that I said it was brownish threw her off track. We has started our plan to kick the habit and had begun the slow down on our smoking so we both thought that it was my lungs clearing up. That was till one day I spit out some phlegm into a Kleenex and my wife saw the color, it wasn’t brownish it was blood.

That sent her into immediate action, she called the family Dr and got an appointment for the next day.

It is now September 8^th, 2011

I went for my appointment with my wife, she had my symptoms written down and we discussed them with the Dr. The Dr ordered a chest x-ray and blood work, and gave me a script for Biaxcin 500 mg 2x daily for 10 days. That was heavy duty anti-biotic so we were all sure it would clear up whatever virus I had. The nice thing about our small town is we live 5 minutes from my Dr.’s and across the street from the hospital so we went over immediately for the x-rays.

During the next week I continuously spit up bloody phlegm, had fevers, severe sweats, just sitting still the sweat would run down my back, arms, legs, face. I was getting more fatigued every day, it got to the point that I would get up in the morning, go for my bathroom routine, have a nap, get up to the bathroom, go back to sleep, eat, sleep, bathroom sleep, coughing and spitting, sweating and exhaustion.

I was having trouble hearing in my left ear as well, this had started one day when at my friend’s pool. I figured I had got water in it and now it was plugged. It was more of an annoyance than anything. I have not worked since September 8^th, 2011.

My wife called for another appointment at the family Dr’s, to get results and to report no change in symptoms. She was becoming very alarmed that I was getting much worse. I have to mention that we had just lost a close friend to cancer late August, yes it was reportedly lung cancer so you can only imagine where our thoughts where while going thru this. We were getting very scared!

September 14, 2011

The Doctor reports that I have a couple of spots showing on my lungs; inconclusive ….he wants an immediate CT scan done on my chest and more blood work. He also ordered about a week’s worth of sputum tests. Each day I was to spit the phlegm into a specimen jar and my wife would take it down to the lab. I was put on 5mg Prednisone, 10 the 1^st day decreasing by 1 until finished. Tylenol #3 was prescribed for the pain as my chest was now hurting every time I coughed; actually it felt like I was ripping my lungs with every cough. I was then referred to an Internist here in Chatham, as well as an ENT.

September 21, 2011

I am at the Internist office going over all my symptoms. He seems to be very stuck on the idea that he must lecture me about my smoking. I have told him about our plan to quit, that we had the patches, gum and our plan and a quit date but I would have to say that most of the time was taken up with the lectures on NO SMOKING!. The Internist has ordered a Bronchoscope, stress test, blood work and an immediate TB test. Back over to the family Doctor’s so the nurse can give me the skin test, I am told to come back the next day so she can read them. At this time my wife and I ask if there are any results from the blood work done on September 15, 2011 (my wife’s birthday). It seems that our Doctor is off with a bad back and no one can read or should I say interpret the results. The nurse tries the best she can and we know some items are out of norm range, but none of us can be sure what they mean.

The skin test show negative results, no TB…for now still waiting for sputum test results.

September 22, 2011 - more blood work, this time for the Internist. Yeah at least some one can read or interpret the results this time.

September 23, 2011- Doppler at the Internist Office, scheduled Bronchoscope for September 28, 2011

All the while I am not feeling at all good. I am sleeping about 20-22 hrs in the day. Just to take a shower takes every bit of energy I have. I am most certain I am going to die. I am pretty sure I have lung cancer, and it is advanced.

The scope is over and went relatively well, not too uncomfortable. The Internist has taken 18 biopsies or scrapings and has sent them away for testing. He went out to speak to my wife and told her he saw no cancer, and was pretty sure there was NO lung involvement. He stated that he did see some bleeding in the nasal cavity and recommended an appointment with an ENT. She was very relieved to hear NO CANCER, but did ask what the cavities where that showed up in his x-rays, she was told that he didn’t know. We had a follow up appointment for Oct. 6^th to get results from the scope.

We walked home (I mentioned we live across the street) and within 10 minutes the Internist office called to reschedule my follow up appointment to October 12^th.. As the Doctor felt there was no lung involvement I really didn’t need the appointment that soon. My wife was furious, she pleaded with the Nurse to help us out. I could hear her say this is a 52 year, healthy (up to 1month ago) male and should not be this sick. Something is terribly wrong and he needs someone to help. The Nurse told my wife the Internist suggested the ENT and when my wife told her the appointment was scheduled for October the 17^th and that I could not wait that long, the nurse promised to see what she could do. The nurse called back right away and got the ENT appointment changed to October 4^th at the hospital.

September 30, 2011 CT scan with contrast die scheduled. Finally, we have been waiting since September 14, and I know that isn’t really long when you are this sick it is very LONG!!!!

October 1 & 2, 2011

My wife wants me to go to the emergency department; our emergency is so well known for 10 hr waits or longer. I cannot do it. I do not have the strength to sit here that long. I am so very exhausted. She wants to drive me to London, the wait is likely to be as long, but she has faith that I would be seen by a specialist and they could figure this out. I turn down her idea (surprised she didn’t fight me harder on that one, I must be very sick or she would have)

I have a pretty rough night, coughing, pain in my chest, breathing is becoming difficult.

October 3^rd, 2011

My wife comes home early from work and I am in my usual spot, where I have been living this past while… my lounge chair. She tells me that enough is enough; she is taking me across the street now to the emergency room. I am hedging a bit and she won’t have it. It is 10am in the morning so we are hoping the emergency room isn’t too full.

The triage nurse takes us in immediately as I am having difficulty breathing, they do the usual test on my vitals and as usual they are all in normal range. That in itself is so frustrating I know I am ill. They run some blood work, do a chest x-ray and put a mask on me and send me to the waiting room. We sit and wait…and wait…I am sleeping sitting up, we try to find a quiet corner , away from others in case I am contagious……after a 4 hr wait they bring me back to evaluate my condition again, the nurses know something is not right but they do not know what it is.

They get me into a gown and on a stretcher and wait for the ER Dr. When he finally arrives he tells me the lung x-ray or more exactly the cavities in my lungs are much worse than the previous x-rays. There is fluid in them, he brings in a specialist. This specialist tells me I have fungal pneumonia and I will be admitted, he further states that I should not have a fungus in my lungs unless I have an underlying immune disease of some sort, he will do test for Aids and go thru the assessment for that. There are no beds on the floor I am going to so they find me a quiet room in the ER for the wait. It is now evening and I have been here since early morning. I start having sweats so bad that I am changing my bed and my gown every hour or so, I just want to sleep and get a good sleep, this is the first time I have been awake for this long in a month or so. It is about 2 in the afternoon the next day before I get a room. I am lucky because I got a private room (they aren’t sure if I am contagious or not. They start gathering blood for more testing and tell me they are working on the medication that will be needed and the dose. They have hooked me up to an saline Iv and now I have to get up every hour to go to the bathroom, all day and all night…I can’t sleep. My wife asks them to cut back on the IV solution and when they do I feel much better, no more hourly trips to the bathroom. The next day in walks the Internist that I saw previously and asks what brings you here, what has changed since I saw you last. In other words I said you were fine why are you here? I could have slugged him, I am sick, I am not getting better, I am getting worse and someone needs to help figure this out! I felt like screaming. Anyway out he walks, never to be seen again, not by me anyway. The next day the hospitalist on duty comes to say they have identified the fungus and will start IV treatment the next day once they have figured out the dose, (meanwhile my wife walks down to the pharmacy and has a talk with them, and they tell her the dose right away) He also mentioned at the time that the Internist has sent away another blood sample and is looking for a rare disease, doesn’t say what it is just says that he must be a country bumpkin and doesn’t know what he is doing. I was put on Fluconazole (very expensive) and sent home 2 days later. It is now October 8^th and I am to follow up with the Internist after 2 weeks and another chest x-ray to see how or if I am clearing up. The last thing my wife does before we leave is to ask for a copy of the CT results and the requisition for a sinus CT that the Internist ordered. When she gets home and starts to read the results, it highly recommends that I see a Respirologist for immediately follow up. She also notes that the blood test ANCA was re-ordered s the first one came back with a high level. She got on the internet and looked that ANCA test up and came up with Wegener’s Granulomatosis, bells and whistles going off, that is it, she is so sure that is what I have, It has listed so many symptoms that I have had going back even 3-4 years…..she is more determined than ever to get me to London.

The Internist is now been made aware that my wife is insisting on a Respirologist so he washes his hands of me. My family Dr. gets the referral for a Respirologist and my appointment is booked for October 27^th.

When we get to London I am in pretty bad shape and in severe pain in my chest (this is new) its take a couple of hours at this clinic and the pain is getting worse, thank god my wife has brought Tylenol 3 with her, that helps ease the pain a bit. The Respirologist has a plan, I am to come back for 7am the next morning to a different hosp to have another bronchoscope done and the nurse tells my wife that I am a pretty sick man. (really, you think)

After the scope the (main man) comes in, explains that the fungal infection is cleared up and that he is sure I have Wegener’s and I am to be admitted. No beds again so they were going to discharge me and have me sit out in the waiting room to wait for a bed, my wife tells them there is no way I can physically manage that and if that’s the case she will have to take me home. The nurses decide that I can stay in my bed at least until 5 when they close, after that who knows. The nurse calls to the Respirology unit every ½ until they find me a bed, it is now about 4:30 pm and off I go to my room.

The next morning the team comes in and verifies that they are 99.9% sure I have Wegner’s. They explain what it is, what the treatment will be, how long it will be. My wife and I breathe a sigh of relief…… It is not a good thing, this disease; but finally someone can identify it, I no longer am made to feel like it is all in my head, someone is actually acknowledging the fact that I am ill.

I am discharged on November the 3^rd and set up with chemo treatments, prednisone therapy as well as a long list of other drugs I will need to counteract the drugs I am on. I am aware that this is going to be a long haul………

Driving home I notice that my vision is very blurred…….I am not seeing things properly at all. Is it the drugs…it can’t be I have only been on prednisone for 3 days and have only had 1 chemo treatment.

And this is how it will be, for the rest of my life……… Symptoms, specialists, drugs, no known plan for my future, no income, no drug plan etc. etc.

I am grateful each day I wake up, this side of the grass, I am grateful that we found support groups that contain the information we need. To know that there are a lot of others with my disease, that there is hope!

Now I am learning to deal with side effects of the drugs, the aftermath of my disease, I cannot do much yet, I am hopeful that once I am weaned off the prednisone I will regain some strength. I am learning how difficult and frustrating it is to deal with the government. I need drug coverage, I need disability money, the list goes on and on…….

Weggie’s Unite was among the first of the sites that we can across, Marta made us feel welcome immediately, she let us know that this will be a life altering disease but can be managed. She provided some of the best clinical information that we have found to this date.

That is my story………………

Last for today is Gary's story...

1. I was born in Edmonton Alberta, and have lived all my life here. I am married, and have three children, two boys and one girl. I grew up in a family of eight children, four of which are brothers and three sisters, all living in Edmonton. My father was born in Poland, and my mother was born in Canada. We were raised as Catholics, and my kids are now raised as Catholics as well. I have played many sports since my teenage years, including baseball, basketball, volleyball at a high level of competition. I have always been fit and in good shape. I currently work for Canada Post as a supervisor, and have been working with them since 1986. Fourteen of those years, I carried mail for Canada Post. I enjoy spending time with my family, all sports and my work. My strongest trait is my competitiveness.

2. In September of 2007, I started with symptoms of a simple cold. Within a week to ten days, the cold was progressing, and I tried several cold remedies, with no results. In early October my condition continued to get worse. At that time, I went to a medi-centre to see if they would be able to perscribe me something to help. I was given anti-biotics, which also did not help. My condition now led to a sinus infection as well as an ear infection. This was now mid October and, another visit to the medi-centre. Where I was prescribed a different antibiotic. This would be approximately four weeks since my first symptoms. At this time, my sinuses and ears were quite infected. I was having trouble breathing through my nose, and hearing was starting to be a problem. This led me to go to the U of A hospital in Edmonton. I was seen in the emergency ward, where they diagnosed a possible viral infection and treated it with more anti-biotics. Once again I did not see results and my condition continued to worsen. I then scheduled an appointment with my family doctor and he was troubled as to what was going on. But he did prescribe me with an even stronger anti-biotic. My condition at that time was severe as I had lost hearing in my left ear, and partially in my right. I could not draw a breath of air through my nose, and could only breathe through my mouth. At that time I was not eating and had lost approximately 40 lbs. I tried the medication that was prescribed by my doctor for about a day, and this led me to vomit for the entire day. At this point in time, my wife schedule another appointment with my family doctor, and at this appointment he instructed her to take me to the emergency ward at the Grey Nuns hospital.

3. When we went to the Grey Nuns hospital, I was admitted immediately due to my vitals being abnormal. The on call doctor at this time started to run many different tests as he had no idea what he was dealing with. This doctor called for an ENT specailist who was on call to have a look at my nose and ears. This doctor confirmed that there was quite a bit of infection in the nose and ears and that this would need to be dealt with immediately. He then proceeded to put draining tubes in my ears, to drain the infection. He was not sure of how they were going to deal with the sinus infection. After this was done the doctors consulted with each other including an intern, who at that time was studying a disease called Wegeners and suggested that this disease could possibly explain my symptoms. I was then sent to the U of A hospital, by ambulance, and immediately admitted there. I stayed there for ten days, undergoing numerous tests and scans and bloodwork, all trying to determine if I did indeed have this unknown disease that the intern had suggested. I had a sinus operation to clear out all of the infection and a biopsy was taken from my nose and sent away to confirm if Wegeners was what we were dealing with.

4. The results came back and it was confirmed that I did indeed have Wegeners. The treatment was started with prednisone and methotrexate as well as folic acid. My disease specialist was Dr. Homik and she had an assistant who's name was Dr. Katz, who eventually became, and still is, my rheumatologist. My ENT doctor is Dr. Ansari. Dr.Katz is the doctor who quarterbacks my treatment and in conjuction with Dr. Ansari, has been treating me ever since I was first diagnosed.

5. Aside from the first six months of prednisone, which we all know is a tough pill to swallow, because of its many side effects, and the weekly injections of methotrexate, I have had been able to handle the drugs quite well. I am concerned though, of the long term effects of using the drugs. I also realize that for the moment this is what is keeping me going and suppressing my disease. One of the side effects that I have been dealing with since my diagnosis is the issues related to my nose. These issues consist of constant nose bleeds (up to three times a day) and also having to constantly clear the nose. I have tried all recommendations from my ENT doctor, but it seems like this is something that I will just have to deal with. As you well know we wake up sick, and we go to bed feeling sick. We just need to get used to it, and begin to take it as the new normal.

6. Since I was first treated I have had one cold and one bout with the flu. My immune system seemed to react as it should, and do the right thing. My disease specialist, Dr. Katz, also diagnosed me in 2009 with the possibly of having Fibromyalgia. He suggested that I do a one week course on how to deal with this. I'm not sure if I do have it or not, but the course did give me important information on how to deal with some of the symptoms of Wegeners. Mainly, it taught me how to relax and prepare myself for sleep, with relaxation skills as well as core skills to help me with aches and pains.

7. Since being diagnosed my life is quite a bit different. As you all know, after getting over the initial shock of this "well-known" disease ( LOL ), things change. I have tried to get back to my normal routine. Of course this is not entirely possible because of the obstacles that are set out for us. I think that the most important thing that I personally have going for me is that I refuse to let this disease hold me back. My fitness level at the time of being diagnosed was probably what saved me and I continually try to keep myself active as I think this is one of the keys with dealing with this disease. I had returned to playing baseball within six months of the diagnosis and just recently I have also returned to playing basketball. The aches and pains from these activities in conjuction with the disease make life challenging at best. Add to that working full-time again, this leads to quite a distraction from the disease. I believe that this is a key tool that I have in dealing with Wegeners. My sports background gives me the "never say die" attitude and challenges me with finding a new way to beat this "oppenent"..... still working on that one. Just getting up and doing something goes a long way, as opposed to sitting there and letting the disease control you.

8. My advice, there is no use crying about it as that changes nothing. IT IS WHAT IT IS. How we deal with it, is entirely up to us. Always challenge the disease and you will be surprised how far you can go with it. For now I'm winning, and maybe I can win long enough for a possible cure.

9. First of all, my family, my wife and kids who deal with this disease as much as I do. My neice, who injects me once a week (for the last four years). My doctors who seem to be amazed at how I deal with this disease. And the intern at the beginning of all of this, for whom I do not have a name, that happened to be reading up on Wegeners, and took a chance in suggesting the possibility of it. I would also like to thank, most recently, Marta and her Weggies Unite website for giving us the much needed support that brings us all together finally.

I have a few more waiting in the sidelines but awaiting permission to put them up here. I hope this helps anyone going through a similar ordeal and please if you have a story and would like to share it for here or for our book project send it to me at madcap01@telusplanet.net . Thank you my Weggie friends for sharing your deep personal and often scary experience in the hope that it can help someone else out there going through the same thing. You are all an inspiration.

Here is Ian's story. He just sent it to me last week and agreed to have it on here before it goes in the book. Thanks Ian for sharing and for showing us that this disease is not one for just people in their 4th or 5th decade of life - as is commonly stated in the literature (it's quite amazing actually if you check out the survey results how many young people are affected by this 'older person's disease)

My name is Ian, and I was born on December 30th, 1994. I did many things the average kid did; go to school, play videogames, hang out with friends, etc. I loved to play tennis, and still do to this day. And of course, I have a very loving family. Overall, i think my personal humor made me the person i am; being funny and optimistic all the time. I know you've heard several stories of adults having WG, but i'm going to give you a different perspective; one of a 16 year old boy.
In the summer of 2011, I started getting skin legions, fatigue, frequent nosebleeds, and headaches. Mostly concerned with the skin legions, since i thought the fatigue was from tennis, i went to see the dermatologist. They said i probably had a mild case of eczema, saying it will just go away if i didn't continue to itch it and used some cream they supplied me with. Feeling better, the days passed on until late August, when my junior year of high school began. Around that time, i started feeling some sever chest pain, getting worse by the day. At first, i thought it was just a pulled chest muscle from playing tennis too much, though eventually it was to the point where every time i inhaled i basically curled up on the floor in pain. On September 4th, a Saturday night, i went to a clinic complaining about the chest pain. They took an X-ray, and came back with some bad news; i had 2 unknown masses in my upper left chest, right below my collar bone. Being rushed over to my community hospital that same night, an CT scan was done, coming up with no new results. Fearing that i had cancer, i was sent to Children's Memorial Hospital in Chicago, about an hour away from where i lived. Arriving there at about 1 in the morning, i got into my bed, and fell asleep.
After a couple days passed of blood results revealing higher level of some liver enzymes and so forth, a lung biopsy was called for to see what these masses were. Having agreed to do the biopsy, i was put out by the a anaesthesiologist, i woke feeling groggy, and wondering what would come of the results. A day or two past, and in came my doctor with some news; apparently i have this disease called Wegener's granulomatosis. The doctor told me that he's never seen this disease in someone as young as i am (we both know its a rare disease, and even rarer amongst adolescents), and suggested that i should play the lottery when i'm of age. This was confirmed by the ENT specialist who also did a biopsy of my throat and mouth, seeing as i had gained some rather painful sores to the point my only intake of food was chocolate milkshakes. Days past, and now they are wanting a liver biopsy since it is about twice the size it should be. But instead of going directly over the liver for a biopsy, they needed to go through my inferior vena cava (the jugular) for the biopsy since a direct biopsy would be extremely risky. My parents and I said no, since we didn't like the idea of a needle to retrieve a liver sample go down my neck and through my body like a snake crawling through the african plains. So they started me on the steroid prednisone, a chemotherapy called retuxinab (i was told this was a newer type of chemo (well not exactly chemo but close enough to call it that) that had a very small chance of me losing my hair) once a week for 4 weeks to reduce my white b cells, bactrim as an antibiotic, as well as calcium, vitamin d, and a nasal ointment called bactriban. After being home for the first time in 12 days, i realize i've lost some weight as long as several things in school.
The school work was a pain, the fact being i was in all honor courses and 2 AP classes. I ended up dropping an AP class and getting soem tutoring, while immediately returning back to school half a day almost everyday to not fall even more behind than i already was. But being back in class made me feel normal again, even though some students thought i had moved, transferred schools, or died somehow. Besides that, there was the chemotherapy and 2 doses of salumedrol (another steroid i'm pretty sure) once a week. I was on 30mg of prednisone a day, along with the other medicine i've already listed.

But wait, my liver and spleen are still enormous! Which is why i eventually did get the liver biopsy after my liver and spleen weren't really returning back to their normal state. But this time, they could do a direct biopsy since it was a little better than before. Again, i tried to fight off the anaesthesia, but eventually succumbed to sleep. Days later, when the results had come back, it turned out i my liver ducts were scarred from PSC liver disease. This is where i start taking a bile thinner called Ursodiol for probably the rest of my life. A couple weeks later, i went in for an MRCP scan to see what ducts were damaged. Luckily, they were mostly smalled ducts, and not the larger ones. I also went in for an Upper and Lower Endoscopy, but thankfully it revealed nothing abnormal.
This was all about 5 months ago, but then came another trip back to the hospital. Over my winter break, on December 17th, 2011, i went back since i had a horrible cough. Another Lung biopsy revealed that my granulomas on my chest were getting smaller and hollow, but also infected with some bacteria, as well as my lungs. This turned out to be a pneumonia, and some antibiotics cleared that up. After 6 more days in the hospital, being release a couple days before Christmas, i was happy to be back out in the world again.
So far, there hasn't been any flare-ups (to my knowledge), and life has been pretty smooth. I definitely see things different now, and hope the best for everyone with hardships. For those who are newly diagnosed with WG, all i can say is the earlier they can catch it, the better of a chance they have to fix it. Meaning if they have these symptoms, or anything peculiar at all, they shouldn't hesitate to contact their doctor for advice.
Mostly, however, I'm extremely grateful for all the love and support i have received and still am to this day. From my family, to my friends, doctors/nurses, and even teachers for being so helpful with getting back to school. Without them, I'm sure I would have lost hope before i even began to climb this mountain of a disease.

5 comments:

mikevanJanuary 20, 2012 at 5:19 PM
Truley enjoyed reading all the stories Marta: Thank-you, book or no book it doesn' t matter the stories are what matter. Isn't it something how they all kind of start out the same, then take so long to get help. How smart we have all become......never again will any of us lay dormant while someone tells us "I do not know", I know that we will push for the answers or for someone to find the answers we need. Our hearts go out to all those suffering or recovering or living with this disease!!!
Mike & Kari
AnonymousJanuary 20, 2012 at 8:19 PM
I would also like to thank Marta for these stories. As Mike's sister, I know that Mike is very ill, but I did not realize how much he is really suffering because Mike is not one to complain. I am so afraid for Mike and I can only imagine how he must really be feeling. I am learning a lot about a disease I had never heard of before. Kari has been our rock through it all! I love you both very much and I pray that God gives you strength and the hope to make it through!
Love, Ruth
BeckyAugust 14, 2012 at 5:10 PM
Thank you all for sharing your stories. I am so grateful that there are others out there that understand what it is like to live with WG. Like you, I am blessed to have people in my life that love and support me, but you all give me something they can't - sanity is what comes to mind. Just knowing that everything we go through is real and even when the tests don't find it or the doctors just aren't sure about it. Hope that makes sense. May you all be blessed with many good days!
UnknownAugust 27, 2013 at 6:49 PM
I also wanted to express my gratitude to those that have went down this journey before me, sharing your stories with all of us as we are newly diagnosed. These stories not only help to understand this crazy life that we find ourselves in now but also to know that we are NOT ALONE NOR ARE WE CRAZY!

Again, thank you to everyone
UnknownAugust 27, 2013 at 6:54 PM
I just wanted to say thank you to all those that have gone before those of us that are newly diagnosed on this journey! Your stories are so amazing and yet so similar ... I find myself researching and reading as much I can get my hands on right now. Although my story began I think many years ago, I was just diagnosed 4/2013 and due to high-risk respiratory involvement, I started treatment 8/16/13.

Once again ... THANK YOU AND PRAYERS FOR STRENGTH EXPRESSED TO ALL FELLOW SUFFERERS OF THIS "LOVELY MESS" WE ALL DEAL WITH.

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