A brief respite from fear
Of total neutrality.
With luck,
Trekking stubborn through this season
Of fatigue, I shall
Patch together a content
Of sorts.

~Black Rook in Rainy Weather – Sylvia Plath~

There was nothing neutral about what was happening to my body. My own immune system had advanced a full-frontal attack on its own muscles. Starting with nausea, it had swiftly progressed to body-crushing fatigue. Last to make its presence known was impossible to ignore pain across my shoulders and upper chest. Three weeks and a multitude of investigations later I would have the diagnosis: Sero-negative Immune Meditated Necrotising Myositis.

Not that having a diagnosis was enough to give my rheumatologist a definitive plan of attack for the battle to come. Instead, it gave a vague idea of the possibilities…the groups of medications that could be used to persuade my body that it was not its own enemy.

Unable to brush my hair or get out of bed due to my hip and shoulder weakness, combined with constant nausea and fatigue that was so intense that my brain felt like it had completely shut down, I was desperate for a quick counterattack on the part of my immune system that had been triggered to attack me. I soon discovered that quick and myositis are two words that are never used together. Everything about this disease takes time…a very long time.

Prednisone was the drug of choice for a rapid initial offensive, but it could also cause damage to what we were trying to salvage – muscles. So, it was in itself a double-edged sword. A few weeks of high-dose prednisone could result in prednisone-induced myopathy…a second cause of weakness to add to the first. The hip muscles are most commonly affected…the same muscles attacked by my myositis.

Because of this it was essential to add in a second medication immediately…one that took longer to take effect, but might eventually take over the immunosuppressant effect of the prednisone. My rheumatologist chose Mycophenolate, a drug used so that transplant patients did not reject their new heart or kidney. Several medications could have been used, though none have been tested in a randomised control trial…which meant choosing one was…unfortunately…random! Well…not completely random. My rheumatologist did have multiple years of clinical experience that he could use to his advantage in guiding his choice.

The first few days of prednisone I was mildly euphoric, if only for the immediate cessation of nausea and the resolution of my muscular pain. This meant that food was no longer utterly repellent, and my aching muscles no longer kept me awake all night. Unfortunately, my fatigue was ongoing because my body decided that waking at 3 am and staring at the ceiling while waiting for the kookaburras to laugh at the rising sun was the perfect way to spend the early hours of the morning.

Four weeks into treatment, I had my first small miracle. I suddenly discovered my hands could reach the top of my head!! This may not seem like a big event, but brushing my hair was a luxury I had never appreciated. This first positive correlated with two other events. I had been instructed to start decreasing my prednisone dose after four weeks. However, my rheumatologist was about to go away for a month’s holiday. He rang me before he left and was pleased with my progress. He said to discuss with my General Practioner when to start decreasing my prednisone – maybe in another week or two.

As a doctor well-versed in researching the latest medical literature, I googled every paper I could find to see if there was any evidence for any given number of weeks.  Not surprisingly, every paper was different, all based on some sort of vague logic but little actual evidence. The first said 4 to 8 weeks, the next 6 to 12 weeks, and another 2 to 4 months.  Lastly…and this one seemed to make the most sense to me… don’t start decreasing the prednisone until you have full ROM against gravity! However, this also seemed to go against the mantra: Don’t stop the prednisone too soon, but also don’t stay on it too long.

Decreasing prednisone is more of an art than a science. Every patient is different. Every disease is different. The only thing the same is the overarching goal of the less prednisone, the better for ameliorating the risks of long-term side effects particularly to bones and heart. This then has to be weighed carefully with the benefit being gained short term in the acute illness…which appears to decrease after four weeks and especially after eight weeks. Then there is the potential to precipitate unpleasant or even dangerous side effects while cutting back the dose, as the prednisone has now suppressed the body’s own natural production of cortisol which then needs time to be nudged back into action.

With my fear of deterioration before my rheumatologist came back from holidays outweighing my fear of the side effects of the prednisone, I ended up staying at 50 mg for eight weeks. I had only minimal improvement in my ability to move over that time, so I probably should have started to decrease it earlier as I had been instructed. However, I would soon find out I had reason to fear, though this was probably only correlated to my decrease in prednisone and not caused by it.

At my next appointment with my rheumatologist I was hoping that I would now be eligible for intravenous immunoglobulins. This would be our third front of attack. No one actually knows how this works in autoimmune disease, but one of the theories is that your own immune system becomes so overwhelmed by the information being thrown at it that it gets confused with what it had been doing, thus giving any muscle left a chance to recover from the constant onslaught. Occasionally, your immune system even forgets what it is fighting and gives up altogether.

I felt incredibly anxious at this appointment, my ongoing tachycardia exacerbated by my prednisone-induced anxiety and insomnia. My heart was pounding in my chest while my brain struggled to connect its neurons in a coherent manner. In the end my brain fortuitously clicked into “doctor mode” and I probably came across as a perfectly sane and logical human being.

Even before I got back to the car, my rheumatologist texted me to say that my application for immunoglobulins had been approved. But while waiting for the infusion to be organised, there was still the task ahead: decreasing my prednisone dose. The following day, I dutifully swallowed 40 mg. The following night, I woke at 1 am. Sleep eluded me for the remainder of the night.

My poor sleep continued and was then joined one week later by chest pain. This seemed to be muscular and initially came and went. The second week this pain changed. My myositis had been complicated by bilateral pulmonary emboli in my first week of treatment. Since then I had always had a small amount of pain in my right lower chest when I took a deep breath. This pain was now worse and present constantly. Could my decrease in prednisone have precipitated more emboli? Maybe I had pain from a flare of my myositis? Did either of those even make sense?

By the end of the week, the pain had become much worse and I sensibly, though reluctantly, returned to the Emergency Department. My repeat Chest CT showed complete resolution of my emboli. Great! However, in the area of concern was now a small cavitation…which to me looked innocuous. I had no idea what this was, as the specialist didn’t add to my fear by saying “abscess”. It wasn’t until I got home and Googled “cavitating lung lesions post-PE” that I realised I now had a rare medical condition that could kill me!

Over the next two weeks I had two hospital admissions, with the eventual outcome of assuming this lung lesion was infected and had formed a small abscess. This had now tipped my body over the edge of its ability to cope with what was being thrown at it. Most likely my decrease in prednisone had only unmasked an infection that had been grumbling away for several weeks. My rheumatologist made it clear to me that decreasing my prednisone dose was the best way of helping my body fight the infection and to do so as quickly as I could.

During the first of those admissions, I would get my loading dose of immunoglobulins. Sitting in the infusion center watching the clear liquid gold drip into my vein was almost a surreal experience…the holy grail of immune disease weaponry circulating through my body to seek and destroy…or at least confuse and confound.

Twenty-four hours later the miracle happened. Standing up to walk across the room, I suddenly noticed my hips felt different. It felt like they were actually moving. Immediately I raised both arms above my head, stood up from a seated position without using my hands, and then burst into tears. It was so wholly unexpected. I had thought it might happen slowly over a week or even take a couple of months, but instead it was almost instantaneous.

Once on antibiotics and immunoglobulins my prednisone tapering went much more smoothly. As my dose decreased, my sleep improved and my anxiety lessened. Though my fatigue continued, I could at least knit again, something I had been too exhausted to do for several weeks. I joked to my husband that if I was ever too unwell to knit, he should take me to hospital.

There was nothing neutral about my body’s relationship with prednisone: it was either love or hate! At the beginning, I loved the immediate onset of lack of pain and nausea. I also enjoyed the initial mild mania, which gave me enough energy to write after lunch, though this was unfortunately short-lived. This was then followed by waking in the early hours of the morning, sometimes even before my (night owl) children had gone to bed! I was now exhausted by lunchtime, napping every afternoon. My anxiety about the unknowns of my disease process was likely worsened by the prednisone…possibly causing me to stress more than I otherwise would have.

The one common side effect I didn’t have from prednisone was weight gain. Before starting treatment, I had lost almost 10% of my body weight. I then continued to struggle not to lose weight even while on high-dose prednisone, resorting to having nuts and yogurt before going to bed. It was only after starting immunoglobulins and then antibiotics for my probable/possible lung abscess that I started to regain the weight I had lost early in my illness.

The first patient to have her Rheumatoid Arthritis treated by the experimental compound “E” was initially euphoric with the relief of her pain but a few months later would have a mental breakdown. She refused to ever try it again, stating that the cure was worse than the disease. This substance, cortisol, would eventually be made into the medication we now know as prednisone. Seventy-five years later it has yet to be bettered, though its side effects mean that the love-hate relationship of patients who take it persists.

As I read about the first patient treated with cortisol, I wondered how disordered my own mental state had been while on prednisone.  I probably lacked insight into what I was really like…and my own lack of decision-making ability…but I realised that I also had to be forgiving of myself…and trust that I will be also be forgiven for mistakes I made when stressed.

The fourth week after my immunoglobulins coincided with my decrease to 25 mg Prednisone. By Friday my fatigue was completely overwhelming me. Was this due to my lack of prednisone, my lack of immunoglobulins, my lung abscess, or was I just depressed…the ups and downs and unknowns of my disease causing me to finally break down?  Probably it was a bit of all of them mixed together, and all I can do is live one day at a time and remember the miracles… “those spasmodic tricks of radiance” …which give me hope for the future…despite the long wait…

Miracles occur.
If you care to call those spasmodic
Tricks of radiance
Miracles. The wait’s begun again,
The long wait…

~Black Rook in Rainy Weather~
Sylvia Plath

Flowchart for Decreasing Prednisone Dose: