“Handle them carefully, for words have more power than atom bombs.”

Pearl Strachan Hurd

The neurologist’s words reverberated around my brain like a bomb. “You aren’t weak; you are just fatigued. IVIGs don’t make you stronger; they just give you more energy.” What he was saying was so bizarre that it felt like the quackery touted by Instagram influencers, guaranteeing to cure any illness with yoga and celery juice, with a paltry $400 for vitamin infusions. I had instead paid $400 for someone to summarily dismiss my symptoms as being unreliable and inconsequential.

I had gone to my appointment naively unprepared to debate my case and argue for better treatment. My appointment three months before had been affirming and reassuring. My fluctuating weakness may have confused my rheumatologist, but my new neurologist assured me that every patient with myositis is different and not to stress. He said my weakness was likely metabolic, which was why my CK was normal. He excluded an overlap neurological autoimmune disease and reassured me about the future.

Over the two years of my illness, I had rarely been this relaxed about a doctor’s appointment, but I had faith that I would be listened to and heard. Usually, I had to resist being forced into a box too small to contain the bizarre range of symptoms my rare diseases managed to produce. This time I had confidence my specialist could cope.

The second relapse of my autoimmune myositis had resolved after increasing my immunosuppressant medication and splitting my IVIGs into infusions every two weeks instead of four. This was followed by two months of good strength and the ability to walk up to two km. But as always, the recurrence of sudden deterioration caught me unawares. The only bright spot was that I only had a two-week wait to discuss the next possible treatment option with my specialist.

However, instead of being calm and relaxed, I should have been anxious and hypervigilant, spending my time analysing the multiple possible meanings of the words I was using. I had little awareness that my wrong use of typical patient language could be so grossly misinterpreted.

I complained of recurrence of severe weakness…I was once again having difficulty walking and was unable to raise my arms over my head. Somehow my specialist heard what to him sounded like fatigue. He told me I was tired, not weak. This stumped me. What had I just said? That was not what I meant. Hadn’t he just examined me and found I was unable to resist downward pressure on my hip and shoulder muscles? Did he think I was faking it?

In vain I tried to push back, trying to explain that my rapid fluctuation in strength was due to my muscles becoming paralysed as my medications wore off, which was then temporarily reversed by my next infusion of IVIGs.

But he wasn’t listening. He followed up by informing me that my IVIGs only gave me energy, so that I felt better. They didn’t make me stronger. I was starting to sink now. I hadn’t come prepared to fight, and I couldn’t wrangle my thoughts fast enough to mount a speedy counterattack.

But my muscles start working again after my IVIGs, I replied. He then stated that they couldn’t possibly work that fast. I knew they could…not just for me, but for many patients. There was an article that examined the multiple modes of action of IVIGs. If they had a rapid onset of improvement, the disease was likely antibody-driven. With these patients, the effect of the IVIGs also wore off as their concentration dropped.

I said that it felt like the Myfortic was no longer working. My T cells were becoming elevated again.  He asked if I had seen an immunologist…insinuating that if I hadn’t, then what I thought wasn’t worth considering.

He ended the appointment saying that there were no other possible treatment options and that he didn’t want to see me again for six months….unless I was unable to walk. Considering that even before I started treatment and was unable to get out of bed or brush my hair I could still walk, this was unlikely.

At first I was resigned, but I quickly became angry. Then I began to make a plan! I would see an immunologist to get their input on possible treatment options. I would continue to see the exercise physiologist to document my progress… or lack thereof. I discovered I could use the recumbent exercise bike and was pleased I could maintain my cardiovascular fitness even while struggling to walk. I could be at peace while waiting if I had a plan. I sent my neurologist an email outlining what my next steps would be. It felt like I was doing his job for him.

What I didn’t tell him was that I saw no possible way that I could continue to trust him to make treatment decisions for me. He had shut me down and not listened to me. It was not worth my time or energy to try to change his mind. From past experience I knew that this was impossible anyway. Specialists never changed their minds, even when they were eventually confronted with incontrovertible proof that they were wrong. I would need to get a new specialist.

Fortunately I already knew who that specialist would be. I had met a neurology professor at the Myositis Roadshow in Brisbane the year before. She had assured me that I was being under-treated and given me advice that enabled me to confidently argue with my rheumatologist and persuade him to increase my medications. I would get my GP to refer me to see her and my husband and I would take a medical holiday to Western Australia.

The dilemma I face is the necessity of “selling” my story rather than “telling” it. I am well able to sell my patients’ stories to the appropriate medical specialist, but that is different to selling my own story. For the first I am the calm and largely dispassionate emergency doctor, trying to get the correct diagnosis and treatment for my patients. In the second, I am the patient, now faced with increasing disability, trying to get a plan of where we could go next. In the first, I have credibility. In the second, I am seen as an unreliable narrator with no insight that my symptoms are psychosomatic instead of physical. And once someone decides you need to see a psychiatrist, all attempts to fight against this look melodramatic.

I may be a bad salesperson, but I am a good witness. I know what I have experienced and what I know to be true. I don’t have an agenda. I am not trying to deceive. I just want to be listened to and believed. The most frustrating thing is their lack of curiosity. They don’t ask questions. They don’t try to work out what is different now from how I was a month ago. I try to tell them, but they don’t have the time to listen. They are not interested in how it feels to inhabit my body and live my life. They just give definitive statements that are so wrong that they are absurd.

So I considered what words I should be using to explain my disability. My muscles feel like they are partially paralysed but that seems a bit too dramatic. Paresis means mild weakness, but my weakness is not mild and that is probably just as likely to be misunderstood. I chatted to a doctor friend who also has myositis, and she says to her doctors that she doesn’t have strength rather than saying she has weakness. Her brain is motivated, but her body can’t do what her brain wants it to do. This does seem to be clearer…the lack of the ability to move is described by an absence of a positive quality rather than the presence of a negative quality.

Our words can be both positive and negative. They have the power to lift people up and to tear them down. They can affirm and encourage, or they can shame and belittle. Believing others’ lived experiences validates them and gives them hope. Refusing to listen results in ongoing trauma and a feeling of hopelessness.

When my doctors don’t believe me, they stop seeing me as a person. Instead, they see me as merely an unsolvable problem, and they hope that I will just eventually give up and go away. If I were depressed, I might just do that, but unfortunately for them and fortunately for me, I am not depressed…and so the fight continues.

My heart be brave, and do not falter so,  
Nor utter more that deep, despairing wail.  
Thy way is very dark and drear I know,  
But do not let thy strength and courage fail;  
For certain as the raven-winged night
Is followed by the bright and blushing morn,  
Thy coming morrow will be clear and bright;  
’Tis darkest when the night is furthest worn.  
Look up, and out, beyond, surrounding clouds,  
And do not in thine own gross darkness grope,  
Rise up, and casting off thy hind’ring shrouds,  
Cling thou to this, and ever inspiring hope:
Tho’ thick the battle and tho’ fierce the fight,
There is a power making for the right.

~Sonnet – James Weldon Johnson~

Reference:

Rapid and reversible responses to IVIG in autoimmune neuromuscular diseases suggest mechanisms of action involving competition with functionally important autoantibodies – Journal of Peripheral Nervous System – Dec 2013

https://pmc.ncbi.nlm.nih.gov/articles/PMC4285221

“We hypothesize that pharmodynamic results characterized by a rapid onset of action, strong dose dependency, and short duration suggest direct competition between IVIG and putative pathologic autoantibodies. In turn, these characteristics imply that the autoantibodies bear primary responsibility for the nerve dysfunction, and that decreasing the autoantibodies ameliorates the symptoms.”

“In contrast to transient relief of symptoms, true remission often requires immunomodulators other than IVIG. Different mechanisms of action of steroids vs. IVIG are also suggested by recent results showing that a higher proportion of CIDP patients treated with high-dose methylprednisolone maintained long-term remission than those treated with IVIG.”

“There has recently been intense interest in the hypothesis that enhanced expression of an inhibitory receptor, FcRIIB, is induced by a small subset of molecules in IVIG. IgG molecules which are fully sialylated are hypothesized to indirectly increase FcRIIB expression, thereby inhibiting inflammatory activity of macrophages. This is proposed to account for the need for high dose IVIG to achieve anti-inflammatory effects, since only a small percentage of its molecules are fully sialylated.”