“Hope” is the thing with feathers –
That perches in the soul –
And sings the tune without the words –
And never stops – at all –

~Hope is the Thing with Feathers – Emily Dickinson~

The Intensive Care Specialist looked at me and then glanced back at the monitor above my head. My blood pressure had been slowly creeping ever higher. Quickly weighing up the possible causes and their treatment options he asked if I was in pain. With my upper arms and shoulders feeling like they were abase I answered in the affirmative, estimating my pain at 8 out of 10.

“You can’t expect to be pain free you know” he said. “Of course not!” I replied with alacrity. It was then he realised that listening to me was likely to be more accurate than looking at me and decided to treat my pain first, before worrying about my blood pressure.

After a bolus of Fentanyl to join a continuous infusion, along with extra doses as needed from my Patient Controlled Anesthesia devise I was soon much more comfortable and my blood pressure steadily trended down to a non-concerning range. He is one of the few doctors who has actually listened to me, instead of reassuring me that I looked well.

On discharge the next morning he joked that I could probably talk under water…1500 micro-grams of Fentanyl over 24 hours failing to significantly sedate me…though I did have excellent pain relief and slept reasonably well considering I was surrounded by chaos in ICU.

A month before I had given up all hope that my Subacute Invasive Aspergillosis would be cured or even controlled by my latest antifungal. All my specialists were in agreement that medical treatment was ideal but they weren’t the ones spending months on end feeling like death warmed up. I was the only one who could logically weigh up the risks and benefits of surgery for me personally.

My general practitioner gave me a referral to see a cardiothoracic surgeon for his opinion on surgical management of my solitary lung lesion. He listened to my story and agreed that twelve months of medical treatment and six months of antifungals was a reasonable trial of medical treatment. I told him I didn’t necessarily expect him to cure me. Just having an definite diagnosis would be enough. That way if I got unwell again, my doctors would at least know the best antibiotics and antifungals to use to treat me.

After multiple negative investigations I often felt that everyone just thought my systemic symptoms were all due to depression. The surgeon was happy to give me a chance of a cure and booked me in for a robotic-assisted wedge resection in two weeks time.

It was the same week as my appointment with the surgeon that my weakness from my myositis dramatically worsened. My IVIGs had been delayed by a day because of a patient requiring urgent treatment and by Wednesday I couldn’t lift my arms over my head and could barely raise my feet off the floor. I hadn’t been this weak since starting IVIGs almost a year before.

Two weeks later my weakness was joined by muscular pain. It was this pain that was causing me problems while in ICU, an intercostal nerve block resulting in little pain from my operative wounds. My arm muscles were complaining vehemently about their intraoperative position of being stretched above my head.

By the next day they had improved significantly and had now settled to a generalised background hum well controlled with panadol, neurofen and some long acting tapentadol. When my surgeon visited me on the afternoon of my second postoperative day he asked when I would like to go home. “Today?” I replied hopefully. He nodded his agreement, and with that I was free to quickly make my escape.

It had been a long time since it felt like my doctors thought I was a sensible and stoic patient…such a lovely feeling to be listened to and believed.

One week later I had my next IG infusion…which for the first time was incredibly uncomfortable due to my constantly aching muscles. My chest wounds had continued to cause me little discomfort. But 24 hours later my muscles were working again and my pain decreasing. What joy!!

My joy was short-lived…three days later my strength was gone and my pain was back. But I had a plan. I was seeing my GP in just over a week. She could then contact my rheumatologist and get his expert opinion. Increasing my immunosuppresants seemed the most logical thing to do, but I was learning that logic sometimes seemed to play little part in many of the decisions of my specialists.

But at least one of my specialists was still logical! Four weeks after my surgery I had an appointment with my Infectious Diseases specialist. It was two months before that he had casually suggested waiting four more months before concluding that medical treatment had been a failure.

Four months that day had felt like a prison sentence of four years and precipitated my decision to obtain a surgical opinion. Now I had good news for him. I had now had four weeks of no systemic septic symptoms and no productive cough.

I had been worried that he would not be impressed with me taking my medical management into my own hands. But instead he was supportive and validated my actions. He would continue to treat me aggressively with twelve months of Posaconazole and would regularly monitor my Aspergillus Antigen in order to pick up any relapse early. I breathed an audible sigh of relief. I had been worried about once again needing to fight to be taken seriously.

He was also happy for me to go back on my full dose of immunosuppresants, stating that getting my myositis under control was very important. I discussed this with my General Practitioner at my appointment the following week, asking her to contact my rheumatologist about our plan, but telling her I was going to increase my dose anyway. I had now been weak for ten weeks, and even increasing my dose now meant a three month wait for it to work.

When my July IVIGs once again only gave me a few days of movement and endurance, my General Practitioner and I made the decision to trial a short course of prednisone. 50 mg worked amazingly well. Within a few hours I was much stronger and able to go on a slow but steady 1000 step walk. I stayed on that dose for two days and then started to decrease it. Each decrease in dose resulted in less ability to move along with more muscular pain.

I felt amazing on prednisone…but was well aware of how annoying it was to wean. I decided to stick with a few days here and there when needed to cope with life. Maybe another round of IVIGs would be all that was needed.

My August IVIGs played out in much the same way as they had in July…four days of increased movement…which I extended with four days of prednisone. This was probably not sustainable and I could hear the annoyance in my rheumatologist’s voice if I had just put myself back on daily prednisone. I could still plead ignorance…or at least desperation to get some sleep without my muscles stabbing me.

Ironically, it was my GP and I who tended to navigate the pros and cons of my various treatment options. She may have had less clinical knowledge of my diseases and their treatments but she had much better knowledge of me and my lived experience of my disease.

When I questioned her as to if I was a “difficult” patient she laughed. This comforted me greatly. Even when I came in sick and fed up I approached it with a dark sense of humour and was always willing to listen to any ideas she had to add. I greatly appreciated her as a sounding board for my ideas (some probably more logical than others), her interest in hearing my stories and her ability to boss me about when I was so sick that my brain had ceased to function.

Most of the time it seemed that my only option with my specialist doctors was mindlessly doing what I was told…which usually involved just waiting and hoping that I would eventually get better. My feeble efforts to explain my degree of disability were usually met with blank stares. I frequently heard that they had never seen anyone like me before. The less enterprising tried to fob me off to another specialist to sort me out. You can blame an autoimmune disease for almost any symptom after all…even a productive cough and pleuritic chest pain apparently!

The most important learning experience for me is the necessity for the patient and the doctor to act as a team, especially when there is a dearth of medical literature that can be used to make a decision. Their job was to give me information and advice, based on their clinical experience. I needed to talk to doctors that I could trust would listen to me and my lived experience of my disease.

I did the research and made the decision to have surgery. I could still relapse after stopping my anti-fungal medication, but I made the right choice for me. Now all I could do was wait and hope…

“Until the day when God shall deign to reveal the future to man, all human wisdom is summed up in these two words -Wait and hope.”

~Count of Monte Cristo – Alexander Dumas~

But those who wait in the Lord
will renew their strength.
They will soar on wings like eagles;
they will run and not grow weary,
they will walk and not be faint.

~Isaiah 40:31~