I write about my rheumatology appointments and I often come away from them upset. Sometimes I come away from them in tears, because while she is sympathetic, I never feel like she can quite understand, or believe, the severity of the pain that I deal with.
Every appointment I tell her that the lumbar spine pain is the worst pain I have, and it literally feels like I have a knife in my back. It is always there, it never goes away. There is no changing positions to relieve it, it doesn’t get better if I lie still, or sit still. It wakes me every night. I can’t roll over in bed, I have to sit up first, change position and lie down again. Most nights I have to sleep flat on my back. It only improves while I am exercising, once I am fully warmed up. Even then it only improves, not goes away, and there are a range of exercises that exacerbate the pain – squats, planks, crunches. I do core work anyway, because I need to strengthen my back muscles, and support my spine, but it hurts like a mofo.
I have become used to it, to a degree, but along with my hips, the back pain is what is forcing me to use a wheelchair, and be unable to walk more than 50 metres or so.
I also have a lot of peripheral arthritis, occasional swelling and redness. She’s never seen severe swelling, but I have photos. She just never seems impressed enough. And she’s so slow with treatments. She’s being careful and preserving the medications I can take because in Australia we are limited to 5 biologicals. She said recently that has changed, its 5 biologicals, and then you have to wait 5 YEARS before you can try another. Ridiculous rules. Makes no sense. So she is being really, really sure that they don’t work before she we change medications. But she makes me say on each biologic for at least 8 months. That’s a very, very long time when the pain is getting worse and worse, damage is being done and each day brings more disability.
But she isn’t a bad rheumatologist. I am a very complicated patient.
Firstly the whole time that I’ve seen her, (almost five years?) I’ve been on prednisone. I am unable to get off prednisone, and my short synacthen test has proven that I have Adrenal Insufficiency. I will likely never get off prednisone. Being on prednisone masks symptoms and confuses the clinical picture.
I came to her with a diagnosis of Mixed Connective Tissue Disease, with Seronegative Rheumatoid Arthritis as the ‘lead’ disease. That’s what we decided to treat.
After proving to her that I had passed the criteria for biologics, she asked me to try Arava for three months, and then I could start my first biological. That was a huge victory to me, my previous rheumy dismissed my disease as mild, and my pain as the result of a low pain tolerance.
I wound up needing to do six months on Arava, just to be sure. Again with the careful, when I needed aggressive.
But by the time I got to her, I had well and truly missed the ‘window of opportunity’ and I now know that I NEVER had a good prognosis. Not even a fair to middling one. Which is why my previous rheumy was happy to get rid of me. And why it was too late and switching medications faster probably would not have helped me.
She started me on Enbrel, and it was fine. No side effects, but no response either. Eight months later she switched me to Humira.
Humira gave me four days of being pain free, and having massive energy. It was amazing. That first shot made me believe I had hit the remission jackpot! I was ecstatic! I ran!!! I cleaned the house from top to bottom. I went to the pub and partied. I shouted from the rooftops how wonderful Humira was.
In fact, I was manic.
Humira then sent me on a roller coaster ride to hell. After every shot I would get that massive burst of energy, which quickly morphed into intense anxiety. It lasted for 10 days, and then I’d crash into depression. Four days later, I would take the shot again, and the whole cycle would start over. It was unbearable, but she made me persevere for 8 long months. But never again did I get that pain free experience, of even much reduction in pain. It helped a little. But it was certainly not worth the mental hell I was experiencing.
Then I started developing neurological symptoms that looked very much like Multiple Sclerosis. She took me off Humira and sent me to a neurologist. Long story short, they didn’t believe it was MS, but they did discover that I’d had several small lacunar strokes, my blood pressure was through the roof (everyone knew that but no one addressed it) and I now have small vessel disease of the brain. That’s a degenerative brain disease, with ever increasing brain lesions and reduced congnitive function. I have problems with memory, balance, dizziness, numbness.
I have neurological deficits, though they appear minor from the outside, they are very much there. But did Humira cause it? Or did something else cause it? No way to know.
I have permanent, painful pins and needles in my hands and feet. Peripheral Neuropathy. They never go away either. Are they neurological deficits? Was it Arava? Do I have diabetes? Nerve compression in my lumbar and cervical spine?
Too hard, no one can figure it out. So no one is treating it. There IS no treatment. I just have to put up with that too.
I had a lot of inflammation in my elbows, and that caused nerve compression. The nerves were compressed for so long that even post-surgery, I haven’t recovered much function. Despite daily physio, I have only 10% function in my left hand and 20% in my right. A year on, there is no hope for improvement.
I have hypermobile joints, or I used to. The osteoarthritis and rheumatoid arthritis damage has caused many of my joints to become stiff and immobile. My elbows and knees are still hypermobile, however.
Both shoulders have synovitis, and I’ve had surgery on my right one to clean out osteophytes as thick as my surgeon’s little finger, and clean up osteoarthritic damage. My ortho saw clear synovitis inside my shoulder, with her own eyes, so there is no doubt that I have rheumatoid arthritis.
There is also synovitis in my hips, visible on ultrasound.
Then my ANA became positive, and Lupus was back on the table. Humira can induce Lupus.
I’ve had a positive ANA in the past, but only low titre. It is speckled pattern, which is highly indicative of lupus/SLE, but also scleroderma, sjogrens and rheumatoid arthritis. So for a while there I met the criteria of Lupus as well.
The last ANA I had a year ago was still positive, speckled pattern, but low titre. So they tend to ignore it, but in my case I think its relevant. Because the neurological symptoms could be lupus attacking my central nervous system. It fits with the results of my lumbar puncture.
So Lupus/SLE is ruled IN.
It could also be CNS vasculitis. Two neuros and a cardiologist suspected that, but I have a negative ANCA, so they ruled it out. But my research says that a negative ANCA, while less common, does not rule out CNS vasculitis.
Then you bring in the Eosinophilic disease. I have allergies, which explains some of it, and Eosinophilic Esophagitis and Gastritis.
But I might also have Churg Strauss, or even Eosinophilic Leukemia. My immunologist says my eosinophils are not high enough, so she doesn’t believe so. The highest my eosinophil count has been is 1.3. She told me to come back if they are steadily over 1.5, or even 2.0. Then she will do the bone marrow biopsy.
That was why she put me through three months of hell and the prednisone taper from hell. And then when it came down to it, she did nothing.
She just bounced me back to my rheumy, who was pretty pissed off, I can tell you. So was I, truth be told. Again, she didn’t realise the hell she put me through.
Then there’s the seronegative Spondyloarthropies. I don’t have psoriasis, and my arthritis is symmetrical. Usually the seronegative Spondyloarthropies (psoriatic arthritis, reactive arthritis, ankylosing spondylitis, and undifferentiated spondylitis) affect the large joints and are not symmetrical. But I have large joints involved, as well as small, almost always symmetrical, and I have had since the beginning. Infact the large joints lit up like a Christmas tree on my very first bone scan, while the inflammation in my hands and feet was mild.
It is quite possible to have both Rheumatoid Arthritis and one of the seronegative spondyloarthropathies. And I do.
For a while my diagnosis was seronegative rheumatoid arthritis with axial (spinal) involvement. And then it became RA and Undifferentiated Spondylitis, because there was inflammation on my SI joints (sacroilitis, hall mark of Ankylosing Spondylitis) shown on MRI, but not on X-ray.
For a diagnosis of Ankylosing spondylitis, you need evidence of erosions on plain radiographs (x-ray). But that radiographic damage is exactly what treatment is trying to avoid. Because that damage is universally acknowledged to cause severe pain and disability.
Yesterday’s x-ray shows Grade 3 sacroiliitis, which means erosions, sclerosis and/or ankylosis are present, and the joint margins have narrowed. That means treatment has failed for me, and the damage is done.
So I now have absolute evidence of, and a certain diagnosis of advanced Ankylosing Spondylitis.
I also have a lot of enthesitis (inflammation of the insertion point where the tendon attaches to the bone), common in spondylitis. I have inflammed eyes regularly, and the Crohn’s type symptoms. All are common in the clinical picture of spondylitis.
So. There’s a lot going on with me. Several rheumatological diseases, neurological symptoms, and eosinophilic disease. I also have coronary microvascular disease, and angina. I have had uveitis and scleritis. I have cataracts, and my vision is deteriorating. I have moderate hearing loss in my left ear and require a hearing aid, and mild loss in my right, no one has explained why that is.
And now we throw cancer into the mix. I have pre-cancerous cells in my cervix, and a complex cyst on my ovary. Before she prescribes any more biologics, we need to know if I have cervical cancer, ovarian cancer, both or neither. The odds are good that its neither, but only the post-surgery pathology can tell for sure.
There is no one disease that explains all of that. And no clear correct treatment.
Just on the rheumatology front alone, there are medications that studies show are better for Lupus/SLE. Hence I’m on plaquenil, even though they are like tic tacs for severe RA. There are others that are better for Ankylosing Spondylitis, i.e. TNF blockers…Humira, Enbrel, Remicade, Cimzia, Simponi. I’ve had two TNF blockers, for little benefit.
I have also tried Orencia (T-cell inhibitor) which gave me 30%. And Xeljanz (JAK inhibitor) which also gave me 30%, but gave me severe liver problems.
So it’s very complicated. Which medication is likely to work best? I have Lupus, RA and Ankylosing Spondylitis. Which one is causing the most damage? Which one do you treat? And then, will that treatment work?
It was never an easy decision, and she is not a bad rheumatologist. She just had some very hard diagnostic decisions to make. She has become as frustrated as I am at times, and we’ve clashed under the pressure of how personally she takes this failure.
And it’s a crap shoot in the end, with which medication will work for which person. The disease criteria and the studies are there to group similar patients and try to find commonalities and thereby predict who might respond to which medication best.
The science is a long way from exact though.
She has done her best.
30% of people don’t respond to any one biological medication. Usually by the third, she has found a winner. She says I’m her only patient who hasn’t responded to any of the four biologics, and her ‘worst’ patient. But I have responded, just not as well as most people. She sees that as failure, and in the beginning, I did too. But now 30% feels like a lot. And I want that.
My only complaint is that she made me stay on some medications for far longer than I wanted to, through horrible side effects, for no benefit. In Australia, that is common, given the 5 strike rule. So even that, I understand. Even if I don’t like it.
So now, in the light of yesterday’s x-ray, I suspect she’ll want to go back to treating the Ankylosing Spondylitis side of things. And that means a TNF blocker. But I will wait and see. I can do nothing until I’m through the hysterectomy.
And then we will decide.
Ankylosing Spondylitis at this stage of the game is well recognised as an excruciatingly painful condition. The spinal damage is disabling, and many people use wheelchairs. Many can’t walk at all. Very few go to gym, and I think my ability to push through that pain confused the picture for many years. Despite my explaining over and over that my activity has reduced dramatically and the kind of exercise I’m capable of is much lighter. Hip arthritis is common, as is referred hip pain from the inflammed and damaged SI joint.
In some ways, I feel justified now, because my x-rays finally bear out what I’ve been saying for years. But the x-rays also mean that there is no chance of the pain ever getting better than this. There is no hope at all. And that’s hard to deal with. I’m an optimist, and my optimism keeps me going through some very, very bad stuff. I can’t be optimistic about this though, damage is done and can’t be undone. My spine will continue to fuse and stiffen and my mobility will get worse and worse.
At least there is no question that I need to move house. Again, the x-rays help on that front, circumstances beyond my control make for easier decision making, even if acting on that decision is hard.
And the x-ray proves that there has been active disease, active inflammation there for years. But they knew that from the MRIs and Spect-CT and bone scans. I’m just a non-responder. And time was wasted while doctors considered whether I have arthritis at all, on the basis of my lack of response. If anyone was at fault, it was my first rheumatologist, for not recognising my disease was severe, and not treating me aggressively with biologics within a year of diagnosis. The window of opportunity. Too late for blame though, it’s pointless, and he also had difficult decisions to make. And I was diagnosed over ten years ago, when biologics were very new here in Australia and VERY hard to get.
Rheumatology is a detective game, with disability as the losing square. Disability is rare, because treatments are much better these days. But some of us still wind up disabled, because there is still a lot more to be learned. We get little sympathy though, because its ‘just arthritis’.
In the end, it’s luck of the draw. I will not dwell on it, and I will not be bitter about it. I will keep on doing everything I can to retain as much mobility and function as I can. I will keep doing spin classes and Core classes, every day that I can. And I will live the best life I can.