The year’s at the spring,
And day’s at the morn;
Morning’s at seven;
The hill-side’s dew-pearl’d;
The lark’s on the wing;
The snail’s on the thorn;
God’s in His heaven—
All’s right with the world!

~Pippa’s Song – Robert Browning~

I was sure I was just being a “drama queen”. After a year of medical stability, I had obviously lost my perspective and was no longer able to distinguish run-of-the-mill muscular pain from a true medical emergency. Once again I was panicking about a symptom that would just settle on its own. It was all probably due to the Zoledronic Acid infusion I had had two days before as osteoporosis prevention. This was well known to cause flu-like symptoms and muscular pain.

By the time I arrived at the emergency department at five in the afternoon, I definitely thought I was overreacting. Yes, I did have chest pain, but it had none of the characteristics that I would have expected from a serious cardiac or even a pulmonary cause.

Initially radiating across my upper chest and back, it was now a squeezing band around my lower ribs. It wasn’t pleuritic and didn’t get worse with movement. It felt muscular, but for the past two hours I had been unable to get into a comfortable position. Just before getting into the car I had vomited, and my pain had almost completely resolved. But I had already made the decision to go to hospital. Despite the improvement in my pain, I still felt woozy and lightheaded, the world spinning confusedly around me.

The Emergency Department was chaotically busy, Thursday afternoons always attracting more attendees keen to sort out their subacute medical emergencies before the weekend. I wasn’t sure I was going to look sick enough to be thought urgent…despite my two hours of 8/10 chest pain.

I sighed inwardly, prepared to wait patiently to put my mind at ease that I was not ignoring an atypical presentation of a heart attack. But my doubts about the validity of my symptoms likely led me to underplay their significance. Much to my surprise, I was quickly taken to Resus, where my normal ECG reassured everyone that I would not require urgent activation of the cardiac catheter lab.

A few hours later my tests were back…all normal apart from my LFTs. That did not excite me particularly as they had been fluctuating up and down for the last month. I had been systemically well over that time and had not been overly obsessed about them. However, for the first time my bilirubin was raised, but I was tired and mostly pain-free…keen to go home and have dinner and go to bed. I did not notice that my lipase was 125…

At home, my dizziness worsened, but I put this down to my brain complaining about the psychedelic effects of the Tapentadol I’d taken to prophylactically prevent a recurrence of pain. I expected to feel much better in the morning.

I did not. I woke up after 8 am and immediately vomited my dinner from the night before. With no preceding nausea, this felt very strange. Head still spinning, I decided to take my medications with a glass of milk. Fifteen minutes later I vomited again. I had no pain, but felt so bizarre that I decided that the only option was to return to ED…once again feeling that I was being overly dramatic!

But I knew that this time I would not be discharged so readily, so I randomly threw some bare essentials into my now dusty overnight bag. This was a skill finely honed over a year of multiple presentations with a lung abscess, but it had been more than a year since my last admission. I did not expect to be seen quickly. Repeat presenters go down the pecking order as they have already had more serious disorders excluded.

I was wrong. My colleagues were well aware of my reputation for underplaying my symptoms; they terrified one of the junior doctors by asking him to see me immediately. He made sure that I didn’t prefer a senior doctor, but I reassured him that I had better confidence in his ultrasound cannulation skills than theirs:)

My repeat bloods showed rapid worsening of my LFTs and an Abdo CT showed an inflamed and thickened gall bladder wall. This confused me because I was now pain-free. An ultrasound and an MRI followed in quick succession. There were possible polyps in my gallbladder and a dilated common bile duct due to some sort of blockage, possibly a stone, though my sonographer husband thought it looked more like sludge.

I still felt quite well, but this was possibly partly due to the rush of adrenaline caused by multiple visits by colleagues and friends. I was also feeling calmer about the situation. Maybe I just had gallstones and would be sent home to see a surgeon in their rooms in a couple of weeks.

Fortunately, the surgeon could read me better than I could. My blood tests had got rapidly worse overnight. After he heard my complicated past history of myositis, immunosuppressants, IVIGs and Invasive Aspergillosis, he gently informed me that he would not be letting me go home. I may have had no fevers with a normal WCC, but I had a very high chance of being or becoming septic. They started me on IV Amoicillin/Clavulanic Acid as a precaution. This was a first for me, as I had never been deemed unwell enough with my lung abscess to be given IV antibiotics (though ironically I had actually felt much sicker then!)

I still thought I had run-of-the-mill gallstones. I wasn’t even tachycardic, which was very unusual for me when unwell. How could I be septic? I was hypertensive, but a BP of 180/100 strikes far less fear into the heart of an emergency doctor than one of 90/50.

I only started to become systemically unwell in the early hours of the following morning. When the gastroenterologist visited me, he initially thought I was tired from lack of sleep, but instead I told him that I felt like crap…which seemed more patient appropriate than telling him I felt septic. As I described my shivering, nausea and dizziness, I could see concern on his face as he carefully weighed up what degree of urgency was required and what he could manage to achieve over the weekend.

What I needed was an ERCP to clear the muck out of my common bile duct. This might then settle the underlying infection enough to decide on the best timing for a cholecystectomy. But even with private health insurance, this was not something easily obtainable on a weekend. He did admit that it was in his skill set, but he hadn’t done one for 21 years, and he wasn’t keen to see if he still had the requisite skills by practising on a doctor-patient.

By Sunday morning I was even sicker. The surgeons consulted with the Infectious Diseases specialist, and my antibiotics were changed to the big guns of Piperacillin/tazobactam, which covered a wide variety of pathogens. I had a few hours of feeling a bit better that evening and hung out at the nurses’ station chatting about the old days and past colleagues, before my chills and dizziness rendered me bedbound and nauseous.

I nagged the nurses overnight about my IV fluid requirements, informing them of my hydration status. I was incredibly thirsty and had low urine output. I had now been unwell for more than 48 hours, and if I was having an anaesthetic the next day while septic, the last thing I needed was to be dehydrated. At first they tried to tell me that I was having the correct amount of fluids…but then they actually stopped and listened and immediately messaged the doctor to increase the rate. My anaesthetist would later thank me for my obsessiveness!

On Monday morning the gastroenterologist visited, and we discussed possible plans to extradite an ERCP before the routine list on Wednesday. The specialist who did them was flying in at midday. He also needed an anaesthetist and an X-ray procedure room. When the resident returned an hour later to quiz me about my fasting status, I realised it was actually going to happen.

By Monday night when the surgeon visited, I was visibly much improved, but he couldn’t see any point in delaying the inevitable and waiting for me to get sicker again. He could fit me in on his list the next day, and I was keen to get rid of the troublesome organ. There wasn’t much need to overanalyse the potential risks and benefits.

The recovery from my surgery was straightforward with little pain, but my septic symptoms continued to come in waves, waking me every morning at five am and lasting for several hours. Slowly these episodes decreased in length and frequency, and eventually the surgeons decided it was safe for me to go home. The worst thing that could happen would be that I’d just have to come back…though they probably have no idea how hard a decision that is to make!

The last miracle of the admission was my General Physician. Bought in to consult on stress dosing of prednisone while acutely septic, she was fascinated by the pathophysiology of my myositis. She was keen to help me get a trial of double-dose IVIGs and was happy to work with my new rheumatologist to facilitate this. Once again I had a glimmer of hope for improvement.

The high point of my admission was the wonderful care and scintillating conversation with my numerous nurses (especially in the middle of the night!). I had a first-year graduate nurse who cared for me with such curiosity and enthusiasm. Every day was a new and exciting adventure for her, and I shared my wisdom of what it was like to be on both sides: doctor and patient. We had both spent a lot of time overseas as children and shared our stories of growing up multicultural, but because of that never fitting in.

I had the pleasure of having the son of a long-time colleague care for me two days in a row in recovery after my anaesthetics. I could vividly remember the day he was “born” as my first day of work in the emergency department more than 30 years before was the day his mum had had the confirmation of his adoption. He was a delight, and I loved hearing about his mum and kids, though I did have to apologise for talking too much in my post-anaesthetic, steroid-filled manic state. He said he never had to worry about a chatty patient!

I was curious how intertwined my gallbladder issues were with my autoimmune disease. Everything about my presentation seemed atypical to me. My inflamed gallbladder wall didn’t seem to be directly correlated with my pain. My septic symptoms only occurred later. The pear-shaped lesions appeared to float in the middle of the sack, and even what was in the duct looked like sludge rather than stone. My LFTs had been randomly elevated for a year.

I figured looking for a paper on acalculous cholangitis in myositis might be stretching it a bit. Still, I found a paper on gallbladder disease in SLE, a far more common autoimmune disease. One of the major pathological processes is vasculitis, which is likely what caused the pulmonary emboli early in the course of my illness. Even in a much more common disease such as SLE, there is very little known about how the gallbladder can be affected.

The other weird thing that happened was three days of constant hypertension and bradycardia. Even after my surgery this would recur during my episodes of feeling hot and cold and shivery. I have often been hypertensive when chatting, but it would always settle quickly if I lay quiet and still. This paradoxical response has been known to happen in sepsis, though the mechanism is a mystery and the treatment unknown.

Hippocrates may have said,  “If you are not your own doctor, you are a fool,” but this is not a foolproof mantra. Hundreds of years later, Sir William Osler said the opposite: “A physician who treats himself has a fool for a patient.”

I had twelve months of struggling with a lung abscess where it was a multitude of physicians who were the ones who had the misplaced optimism. My bloods were normal. I had no fevers. I looked well. I would eventually get better. I eventually had to take the advice of Hippocrates and make my own decisions. I decided that the benefits of surgery to remove the abscess were worth the risks.  Forty-eight hours after my surgery all my systemic symptoms had dissipated.

Because of this experience my greatest fear was my story not being believed…but ironically it was me who didn’t believe what my own body was desperately trying to tell me. My misplaced optimism that for once my body would behave in a sensible manner meant that I headed down what seemed like the logical diagnostic path of a new medication side effect far too early.

Sometimes with myositis, a new disease process can become entwined with the old, but it is always important to keep an open mind and tell your story to doctors who may be able to see the bigger picture. We know our diseases. We know our bodies. But we don’t know everything! Doctors have a lot of learned knowledge, but they don’t know everything either. All of us can have misplaced optimism, as well as pessimism…depending on our individual personality traits! Listening and learning are important for everyone. I may be an optimist by temperament, but I need to learn to treat my body with the pessimism it deserves!

“A physician who treats himself has a fool for a patient.”

~Sir William Osler~

Trust in the Lord with all your heart;
do not depend on your own understanding.
Seek his will in all you do,
and he will show you which path to take.

~Proverbs 3:5, 6~

References:

1. Diseases of the Gallbladder and the Biliary Tree in Systemic Lupus Erythematosus: Review of Clinical Presentation, Diagnosis and Management
https://journals.sagepub.com/doi/full/10.1177/09733698241300754

2 Autoimmune Disorders of the Liver and Biliary Tract
https://pubs.rsna.org/doi/full/10.1148/rg.240126

3. Sepsis-related hypertensive response: friend or foe?
https://pmc.ncbi.nlm.nih.gov/articles/PMC4039975/