Steroids,chemotherapy and living life with GPA

These two pictures of me were taken on the 12th of August - Mother's Day in Thailand. It was probably a few days before I first started having the first of my strange symptoms - unbearable pain under my feet that came and went for a few days. At the sign of the first symptom, I presumed that it had something to do with the kind of shoes I was wearing, and possibly the amount of running that I was doing, as I was training for a 10km race that I was hoping to do well in. I went to a doctor and his diagnoses seemed to be in agreement with my thoughts. Honestly, who could ever have known that it was anything more than that?

Two days later when I started having pain in my other joints, I started to question whether it was something more, and took to Google trying to find every possible diagnoses so that when I went back to the doctor at least I would be armed with some more information. Google is a dangerous place for people exhibiting strange symptoms. You could go from a healthy individual to someone who is convinced that they are dying in a matter of minutes. I however, had no idea that what I did have actually was much more serious than what any of the symptoms were showing at that point and now that I know that I have GPA (Granulomatosis with polyangiitis or Wegener's Disease), I sure am thankful for all the information that I can find on the internet that most of the doctors here cannot tell me, because they have never actually met anyone with GPA. When I was being seen by seven different specialists at a private hospital and none of them had any idea what was wrong with me, I have to say that it was a very scary position to be in. To be told that the hospital can no longer treat you because they have no idea what is wrong with you, and you need to be transferred elsewhere is pretty frightening. 

Through numerous miracles in the span of 48 hours, I had a diagnoses, and a doctor in my very own town who could treat me. She is the only Rheumatologist in the entire town and had just been transferred from the best government hospital in Bangkok six weeks prior to my illness. My blood tests that had been sent off to Bangkok a few days earlier came back just in time, and the doctor returned to work from a seminar that she had been attending in India the same morning as the blood results came in. At this point I was already on a ventilator and a feeding tube, receiving numerous blood transfusions and my lungs were getting worse by the minute. Once the diagnoses was made, the doctors started plasmaphesresis, substituting the plasma in my blood to rid my body of some of the antibodies that had turned on my own body and were attacking it. They did this for five days in a row. I was already on steroids, but my dose was increased significantly, and once I was off of the ventilator, I was informed that I would start Chemotherapy and that it would begin the next day.

The thing about having a disease like GPA, is that when it progresses so quickly, you don't really get to think about any of the treatment before it begins. It's not like you really have a choice in the matter - the honest truth most of the time is have the treatment and you live, don't have the treatment and you may not be around for very much longer. When I was first put on the ventilator, the nurses and doctor asked me a number of times whether I wanted to have a ventilator put in. I was watching my oxygen numbers drop rapidly, knowing that I couldn't breath and was only getting worse, but at that point I just needed someone to make the decision for me. These are the kinds of situations you never think about and being asked whether it was something I wanted or not was very difficult when I felt so uninformed and powerless. If the doctors hadn't made the decision for me, I am pretty sure I wouldn't have made it until the next morning.

I have learnt through all this then that sometimes it is better to be uninformed when treatment like this begins. While I knew what both steroids and chemotherapy were, I didn't know enough about them to know what the side effects would be like. Had I known, perhaps I would have hesitated to allow the doctor to give me both - even though I need them to treat the disease. Most people that I have met online that have GPA hate being on steroids, but I would say that almost everyone says the same thing - they hate the side effects and wish they could get off the drug,  but they are happy that they are still alive.

So here is some of what life is like on steroids, and chemotherapy - which is what life with GPA looks like for me right now. I was started on a very high dosage of Prednisone (steroids) when I was in the hospital. By the time I left the hospital I was taking 50mg of steroids daily which I presume was less than what the doctor started me on, but I can't say for sure. The side effects were nothing that I ever could have imagined. I had been told by the doctor that I may get a 'moon face', one of the side effects of prednisone, but she didn't tell me that I would be hungry constantly, not able to sleep for longer than 45 minutes at a time, constantly sweating, having heart pulpitations, grow a weird fat deposit on the back of my neck, and find it very difficult to control my temper. There are risks of osteoporosis, damage to my teeth, developing diabetes and high blood pressure and going into early menopause - but I won't know until I have been on the drug long enough for it to affect me in any of those ways.

Definitely the hardest two side effects to deal with have for now have been the sleeplessness and the moon face. I am not and have never been someone who cares that much about my appearance, but it was so odd to watch my face changing before my eyes in the mirror each day. People immediately started to comment on my face every time I left the house. I decided that it would be interesting to take a picture every couple of days and put them together to compare them. This is the progression in around 6 weeks, from just before being released from the hospital when I had already been on Prednisone for a number of days, to now when I have been on it for more than a month and a half. Admittedly, I don't look bad and probably look healthier with some cheeks anyway, as I have always had a very long, thin face. But watching my face change so fast has been quite surreal.

I have already had my prednisone dose reduced a number of times during this 6 week period and am pleased to hear that most people lose the moon face after their dose gets lower than a certain amount. I am looking forward to that day, when I can sleep at night without waking up in a puddle of sweat every few hours, and I can leave the house without people commenting on my suddenly very round facial features. But for now, I am like many other people with GPA, happy that the prednisone is keeping me mostly symptom free and that I am alive, breathing with my own lungs and walking pain free on my own legs for the most part.

Chemotherapy for me was more of a psychological hurdle than a physical one. The physical side effects are there, but I think there is a certain stigma about receiving chemo that makes one want to think twice before agreeing to the treatment. Again, I was thankful that my doctor didn't give me a choice. There was no time to think about whether I would or wouldn't have the treatment, or whether there was even an option not to have it. I didn't ask too much about the side effects, if the doctor thought that it would keep me alive and put me into remission, I was happy to go along with pretty much anything she suggested. I had just come off a few days on a ventilator and was finally breathing on my own, anything that the doctor thought would keep me breathing without help from pipes and machines seemed like a good option at that point.
I was told in the morning that I would have the treatment and had my first treatment that afternoon. It was only when I was having the treatment that I began thinking about the side effects that I may have. Would I feel sick? Would I lose my hair? Would I one day get cancer from the chemicals that were being injected into my body? I don't know the answer to any of these questions, but again, I can say that I am happy that the drugs are helping to keep me alive. I may still lose my hair and the chemicals may affect my body one day in the future in a serious way, but for now I am alive and getting better, and for that I am grateful.

At my last check up with the doctor, she was amazed at both my blood results and my lung x-ray. I am walking a journey of faith and believe that God is healing my body every day. I honestly don't know how much of a role all the medicine has had to play in the healing process, but I do know that every day I get up and try to do all that I can to follow God's leading in how much I take on. Six weeks ago with the state of my lungs, I was pretty sure that I would never run again, which was devastating to me having been a runner all my life. I have been walking every day, and praying that my lungs would recover completely. When the doctor looked at my x-ray last week, her words were: 'Your lung x-ray looks like a normal person's lungs'. She then gave me the go-ahead to run or swim as I feel able. Between chemo treatments, I feel well enough to exercise most days and I am pain free the majority of the time. This is a journey that I take slowly and with caution, but the joy in being able to do something that you really love and thought you would never do again is an experience in and of itself that I feel privileged to have had.

So what does life look like with GPA - some days I am tired, other days I feel fine. It is still strange to think that I live with a chronic illness and to be aware of the fact that my body is fighting a battle against itself. It feels bizarre to know that I have a disease that most people have never heard of, and that just a few weeks ago, I myself had never heard of. It is frightening to think that I could end up back in the ICU in a matter of days or hours just by picking up an infection that my body would ordinarily fight on it's own. GPA is not a disease that goes away, and so even going into remission, which is what I am hoping for, there will always be the chance of the disease flaring again, whether that is in a couple of weeks or many years in the future. For now though, I am taking each day as it comes. I am 3 treatments into my 6 treatments of chemotherapy, and if all goes well I will be finished by the end of the year. I have significantly reduced my dosage of prednisone and am feeling well on the lower dose. I have gained enough strength to do most of what I need to get done on any one day, and have a wonderful understanding husband who does everything that I cannot do, or finds someone else who can do it for me.

Life with GPA has given me a lot of perspective. I have thought a lot about things I have never had to think about before. I will leave the philosophical side of things for another day and another blog post - there is much to be learnt from the challenges that life brings us, and I know I am only just beginning.