Wegeners

The story of Kay

When I was 21 my body started hurting all the time. I couldn’t do the simplest of tasks and my husband had to help dress me. I cut off all my hair because lifting my arms to brush my hair hurt too much. I started coughing up blood and my GP said that was a result of chronic bronchitis due to smoking. I suffered for months with no explanation for my pain. Practitioners guessed Fibromyalgia, or just thought it was me overreacting.  After about 5 months of unbearable pain where I would wake up every night crying or lie in bed screaming my doctor finally did a blood test and decided I had Rheumatoid Arthritis.   

They continued to ignore the fact that I was coughing up blood even though I had quit smoking for several months. I finally was referred to a Rheumatologist who was going to put me on methotrexate to lower my immune system but didn’t seem to feel there was any hurry and didn’t give me any information on how RA could affect my body. She just kept telling me everyone was different. She didn’t even really explain what methotrexate was or what Rheumatoid Arthritis was. She didn’t give me anything to help with my pain either. I was trying acupuncture which was he’ll because lying still was excruciating. I ended up popping Ibuprofen all day every day.

  I got so sick I couldn’t eat, my stomach would have really sharp pains every time I tried. My cough was getting worse and the frequency of blood I was coughing up was more. I hurt so bad I wanted to kill myself but I didn’t have the strength to get up and do it. My mother-in-law and best friend came to stay with my husband and me to help with our dog grooming business because I wasn’t able to work. My mother-in-law told me I needed to go back to the doctor. I didn’t want to because they weren’t helping me but she insisted. I saw a different doctor in my GP’s medical group and she immediately noticed I was extremely pale. She did a blood test and found I was extremely anemic and told me to immediately go to the hospital to get a blood transfusion.

  I was terrified. I didn’t know what was happening to my body. I didn’t know if this was something that happened to people with RA or not because my original rheumatologist gave me no information. I was immediately sorted to the hospital and they quarantined me thinking I could have Tuberculosis since I had been coughing up blood. I was given two blood transfusions and then they started the tests. I came up negative for Tuberculosis.
  They did imaging of my lungs and found I had several growths in my lungs. The first thing they said was lung cancer. That’s a word you never want to hear, especially at 21 years old. 

  To find out if the lumps in my lungs were cancer or not they wanted to do a biopsy. They explained that they would stick a needle through my chest into my lung to get samples. As they explained the possibility of lung collapse I broke down. All of this was so scary. I hated being in the hospital. I was constantly being stabbed with needles. One day they couldn’t get a vein because my veins are terrible and they brought in ten different people who were unable to get an IV in. They ended up putting a picc line into my arm. I loved it because they were able to give meds and take blood easily, but there was one nurse who didn’t clean it and I was afraid it would get infected and I could get sepsis. I had a friend who had died from this very thing in a hospital. My mom complained and she wasn’t my nurse after that.
  The lung biopsy came back as negative for cancer which was a relief but also not because it meant there was something else wrong with me and no one knew what. 

They did an endoscopy to try to figure out my stomach pain and general anesthesia doesn’t work on me so I woke up in the middle of it. It was terrifying and traumatic. Before getting the tube out of my throat and telling me to keep calm they did see I had an ulcer. My Rheumatologist came in to see me and scolded me for taking ibuprofen because that was what was causing the ulcer, especially on methotrexate, but she hadn’t told me this when I originally saw her.
  The doctor in charge of me in the hospital decided to bring in another Rheumatologist for a second opinion. His name is Dr. Robert Fox and he is one of the best rheumatologists in the country. He actually gave me answers about Rheumatoid Arthritis and what it does. He started me on high doses of Prednisone and for the first time in six months I wasn’t in pain. My husband asked if I could be his patient from then on. 

  I was in the hospital for eight days over Christmas. They decided I was well enough to be released even though we still didn’t have a diagnosis which scared me. Once I was released they had me go see a pulmonologist who told me the results of all these tests came up as something called Wegeners Granulomatosis, but that he didn’t know enough about it and I needed to see Dr. Fox to get more info and start treatment. My Dad is in the biochem industry so he immediately went and researched. He sat me down and said what he read was people with Wegeners die with five to twenty years…
  The wait time to see Dr. Fox is months because he is the best, but I needed to get in immediately. My mom went into the office and yelled at them and demanded they get me in because “her daughter could die!” They got me an appointment within the week. 

  My parents and husband came with me to my first appointment with Dr. Fox and he found out what my Dad did for work and told me to ignore anything he’d read because treatment for Wegeners was getting much better and I had what was then a “limited” version where my kidneys were not involved which is what usually kills people with Wegeners. He said that Wegeners Granulomatosis was an extremely rare vascular autoimmune disease. It causes extreme inflammation of the blood cells which is where my pain was from and the growths in my lungs were granulomas which is basically pockets of inflammation. There are fewer than 200,000 cases of Wegeners per year.

  I was given chemotherapy once a month for six months. It was horrible. I was so sick for a week after and the medicine they give you does absolutely nothing to help the nausea. The only thing they gave me any relief was marijuana, which is when I really started supporting the medical use of it. After six months of chemo I was put back on high doses of Prednisone for six months. I gained tons of weight, I was already an insomniac and it made it so much worse. I could go three days without sleeping even though I was on three different major medications to help me sleep. It made me deteriorate mentally. My anxiety was horrible. 

  After the six months Dr. Fox had great news. We didn’t have to go back to another half year of chemo because I was in “remission” where I just needed to be on immunosuppressants for the rest of my life. I was one of the lucky ones to respond so quickly to the treatment. My lungs were clear of the granulomas. 

  I’ve been seeing Dr. Fox every three months for the past 14 years and I do have minor flare ups where I really hurt but I had been lucky that my Wegeners stayed under control.  They changed the name of Wegeners to GPA (Granulomatosis with polyangiitis), because Wegener was a Nazi and they didn’t want him to be given credit. But I feel it is perfect for this disease to be named after a Nazi and everyone who has it still calls it Wegeners Granulomatosis.

  This last October my Wegeners relapses because I was going through a lot of dress in my life. My gums started swelling and having little wounds and were super painful where I couldn’t eat. My body was hurring, but I thought it was just from my physical job. I was coughing again but not coughing up blood so I didn’t know it was wegeners related and went to a GP. He told me to talk to Dr. Fox because he didn’t know what it was. I sent a picture of my gums to Dr. Fox and he said it was what is called Strawberry Gums which is a way some people are diagnosed with Wegeners. He also had me get a chest x-ray for my cough which showed one giant 8.3 cm mass in my right lung.

  There is a new treatment for Wegeners called Rituxan which is given as an infusion kind of like chemo but it is more targeted rather than completely wiping out a person’s immune system. I don’t really know enough about it to explain it but it can have scary side effects. Dr. Fox wasn’t able to prescribe me a Rituxan infusion beci he was out of my HMO and my GP was dragging his feet so Dr. Fox told me to go get admitted to the hospital so I wouldn’t die. 

  They weren’t convinced it was wegeners because usually wegeners is multiple granulomas in the lungs (although there has been at least one other case of a single mass) and they ignored my gums. It was a nightmare of ten days of keeping me there doing unnecessary tests to finally confirm it was a Wegeners flare up. They did a bronchoscopy which was inconclusive so they did a needle biopsy which took 4 days to get results back because it was over the weekend which did confirm it was Wegeners which I had been telling them for nine days. They finally gave me Rituxan and high doses of Prednisone and sent me home. My gums immediately got better and the lung mass has gone down by half. Dr. Fox said my inflammation levels were record breaking, but those levels are back to normal. Now I’m just trying to get off of Prednisone safely because it is causing a lot of anxiety and depression and memory loss and I feel like I’m going insane. Hopefully I can get back to normal soon and hopefully I’ll get at least another 14 years of this disease “being quiet” which is what we now call “remission”.