It is now six years since I began steroid treatment for Addison’s disease – primary adrenal insuffiency. In October, my kids brought home a hacking cough with fever; we all spent a week on the couch with a round of antibiotic. The kids got better and went back to school and I went back to the doctor for some antibiotic for myself. I was coughing so hard my ribs ached and I had a bad few days with muscle spasms round the right side of my rib cage.

Monday morning

After three weeks with a fever on double dose hydrocortisone, plus two weeks of antibiotic, I woke up on a Monday morning with what felt like very swollen lymph nodes all down my right side, a stiff neck and the temperature was back. I was thinking the hacking cough and fever had turned to pneumonia – or possibly bubonic plague – so packed a hospital bag while waiting for the GP appointment to be confirmed.

The GP touched the golfball sized lump in my right armpit tenderly, announced: It’s not your lymph node. That’s air. You’ve got surgical emphysema. I have coughed so hard I had popped a lung. He got on the phone to medical admissions at the nearest hospital. I got out my mobile to tell the husband he had three hours to get home from the office and pick the kids up from school.

My local five-star hotel

My GP had done well on the hospital admission. I was x-rayed straight away at A & E, then there was a pause while his referral letter was mislaid, retrieved, and we all retired to the lobby for a fire alarm. Dr Skinny, the chest registrar, turned up to say that I had a partially collapsed lung; he was there to put in a chest drain to allow it to reflate. He led me round the corner and parked me in a room with what looked like a dentist’s chair, returning a short while later with a pile of blood sampling materials.

While he was drawing blood, I jokingly told him the story of the paramedic with Addison’s, who had to insert her own i/v line by torchlight when she got sick while working at a remote Thai mission station. Test tubes duly dispatched, he returned with another clutch of kit. The chest drain is to be done here, on the spot.

Nervously, I asked what sort of anaesthetic he would be using. Local, he answered. By now I was stretched out over the dentist’s chair and a bedside table, ribcage suitably exposed.

I was thinking, I might just need a bit more steroid cover for this one. Can you pass me my handbag? And the water bottle with it? I asked the nurse.

Opening up my hydrocortisone supply I hesitated: would 10mg be enough? Then I thought, better safe than sorry, and swallowed 20mg.

As things turned out, I was lucky I had taken the 20mg. Inserting a length of plastic pipe through the ribcage into the lining of the lung requires some physical force, because there is a certain amount of muscle strength in the diaphragm. Dr Skinny commented cheerfully that it didn’t seem to be as painful for me as a lot of people. There were a few blood stains on my trousers as a badge of honour.
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I felt pretty rough afterwards and took another 20mg hydrocortisone as soon as Dr Skinny and the nurse left the room. My husband and children arrived. They had been waiting outside long enough to hear me say Ow and Ouch loudly a few times, and were relieved to see me looking reasonably coherent. I had the next x-ray, and we all went up to the ward together.

It was clear I could not go home until the chest drain, externally connected via a length of plastic piping to a water bottle with an underwater seal – which I had to carry around in one hand, keeping it below waist height at all times – could come out again.

Me and Dr Grumpy

Up on the ward, Dr Skinny reappeared with a consultant in a suit and a tired, end-of-day look. You’ve got Addison’s, what’s your dose? he demanded. Dr Grumpy was very concerned that I might have popped a lung because my steroid dose was too high and had given me crumbly bones, so that just the impact of coughing hard made me fracture a rib.

Awkwardly for me, Dr Grumpy did not see why I wanted to keep my medication supply and administer my own while an inpatient. It’s simply never done that way, you get your medication as part of the ward round.

The last time I had been in hospital was for my caesarean, five years previously. Then, the medical team on the maternity ward had told me they thought it was best if I kept my medication and took it myself, which I duly did. And I had heard enough from other members of the ADSHG about delays in getting their hydrocortisone as part of the ward round to know that I needed to stand my ground.

I need to have my hydrocortisone on waking. If I have to wait for the ward round, I will spend the first two hours of the day in a state of severe steroid deprivation. And that will impede my recovery.

Dr Grumpy agreed that I could keep my own medication. He decided that, in light of the fever, I should stay at double dose hydrocortisone until the chest drain came out, with antibiotic cover for the same period. Then he left. It wasn’t until the next day I realised he had not enquired as to my steroid cover for the surgery.

Neither had anyone done any post-operative observations of my blood pressure or temperature. The nurse checked them later that evening as part of the standard ward-round observations, but no one had remembered the need to monitor these post-operatively for an adrenal patient. Not to worry, if anything, my blood pressure was too high. Several days later I realised that the large amount of air that had leaked out of my lung into the surrounding tissues was probably responsible for the elevated blood pressure. Or it could have been stress (just joking). Once I got home again my blood pressure went back to normal.

Dr Grumpy came to see me on the ward round next morning. I asked him what my blood tests from the day before looked like; my sodium and potassium were okay, both in the lowest quartile. These low-end numbers probably reflected the diarrhoea the family had shared at the weekend along with our flu bugs. So my high blood pressure was not caused by sodium retention. This suggested I did not need to adjust the fludrocortisone to counter the high blood pressure.

Angels of the night

I settled down in my new home for the night. It was to be a bad night. I had a lot of pain on my right side and by 2 am was asking an angel of the night for some morphine. Even with the morphine I had to sit up every 60 minutes or so to relieve the pressure on my right side.

At 7am I sat up to take my morning hydrocortisone and discovered that – less than 60 minutes previously – another angel of the night had removed my water jug. But it was okay, because they had left me the squeegee water bottle I brought in with me, so I could still swallow my hydrocortisone.

Bones, charts and x-rays

Fast forward 24 hours when I was feeling a little brighter and just the senior house officer, Dr Bashful, came to check me on the ward round. He had enough time to go through the x-rays with me and I discovered that the severe right-sided rib pain I had experienced on my first night was actually caused by my arthritis and nowhere near the site of my fracture, lung puncture or surgery. Looking at the location of the fracture on the x-ray prompted me to recall that the night before I first experienced significant rib pain, my five-year old son and I had been playing a tickling game on the bed. He landed his knee in my ribs at about the fracture site. Then the following morning I had a good cough in the shower to clear my lungs and promptly developed painful muscle spasms around my rib cage.

Probably at that point I popped the hairline fracture my son’s knee had indented in my rib the night before. After which I leaked air for three days and began to look more like the Michelin man before turning up at the GP.

I was still running a low-grade fever which threatened to escalate whenever my steroid levels went down. The fever made me light-headed and I wondered whether it was significant enough to merit moving up to intravenous antibiotic. Then I decided to stick with double dose hydrocortisone plus oral antibiotic for a while longer before I called it to the attention of the medical staff. A cannula in my wrist would be a complete pain on top of the chest drain. The fever never really resolved until the chest drain came out.

Hitting the gas

Three days after the chest drain went in my breathing was clear but I still looked like the Michelin man, with only a modest reduction in the sponginess of my neck. I finally met Dr Shy, the chest consultant, who was also convinced I had crumbly bones from hitting the steroids too hard [1]. He suggested putting me on oxygen to see if that helped the resorption rate.

Over the next four days I was to spend as much time as possible breathing in oxygen through a mask (including overnight, until it slipped off in my sleep). Now I had two lengths of plastic piping curling off the bed, one to the oxygen, the other to the water bottle on the floor attached to my chest drain.

The oxygen did radically speed up my slimming trick so that my rib cage reappeared. In fact, I was to lose weight appreciably during my hospital stay, so that in terms of body mass area I should strictly have been looking to get my hydrocortisone dose a little lower [2]. But not while I was on double dose for a fever. And skinny did not mean glamorous.

Michelin man

Looking in the mirror in the ward toilets I could now appreciate just how like the Michelin man I had become. A thick neck worthy of any hypothyroid patient and a bloated tummy to match a Cushing’s patient. Except that when you poked it, my body fat felt spongy and squelched audibly as the air moved under the skin. I had my own built-in whoopee cushion.

There was no room in my inflated tummy for a standard-sized meal and I got intestinal cramps as anything moved through. Then, 48 hours after I had the drain put in, the cramps got worse and I vomited up my lunch. It was starting to look like gastro-enteritis. I immediately swallowed an extra 10mg and kept that down. But the nausea and cramps were getting worse, so I asked Miss Nimble, the nurse, for an anti-nausea injection. She gave me a prompt stab in the bottom with some cyclizine (an anti-histamine).

The nausea and cramps stayed with me through the night and I sat up to take 10mg hydrocortisone with a swig of water from my squeegee bottle every few hours to control it. (Hospital procedure meant they still took the water jugs away in the night). But I did not vomit again. I slept a lot for the next few days.

Miss Nimble and I repeated this sequence with the stab of cyclizine in the bottom the following evening. This time the nausea was not as bad and I only needed a small amount of extra hydrocortisone. It was never clear if this was Addison’s nausea or gastro-enteritis.

The pharmacist’s party bag

By now I had chewed my way through quite a lot of the supply of hydrocortisone I brought in with me, so I was grateful for the outcome of my final run-in with the hospital over my medication. The nurses were all comfortable with the fact that I administered my own endocrine medication, but the pharmacist who turned up on the Wednesday couldn’t handle it. Mrs Stickler was undeterred by my assurance that Dr Grumpy the endocrine consultant had approved the self-medication regime.

I was still in the fog of a low-grade fever, a developing case of abdominal cramps and nausea, and the discomfort that goes with a length of plastic pipe nestling in my chest cavity. Plus by now I had had a chance to look up my copy of the Owner’s Manual [3], to find that the recommended level of hydrocortisone cover for the type of surgery I had is a 100mg intra-muscular injection.

So when she asked: Would it be all right if I just look at your medication? To check that it’s all in date and correct, my response was more prickly than it might otherwise have been.

No, I said, because it’s mine. And you can’t check the expiry dates because it’s not in the blister packs any more, it’s all in my travel kit. Then I added, I wouldn’t mind so much, if it weren’t for the fact that I had to self-medicate through the surgery. And no one even checked my blood pressure afterwards. You can’t expect me to hand it over after that, I said.

She nodded sympathetically. Tell you what I’ll do, she said. I will re-issue you with hospital medication for your stay, so I am satisfied all your medication is correct, and you can keep it and take it yourself.

We went through my regime and when I explained that I had taken my first hydrocortisone on waking at 6am that morning she nodded again. That’s three hours before the ward round, she commented thoughtfully.

So Mrs Stickler brought me back a party bag with more of everything, including a supply of pain-relief for the chest drain.

Meet Mrs Pneumothorax

None of the ward staff ever enquired why I needed my endocrine medication, and even Miss Nimble, the nurse, did not enquire how the sickness and fluid loss could affect me [4]. I realised she was unaware I was increasing my medication to manage the vomiting and nausea, but did not have the energy to explain it to her.

Every medical condition has a label and the term for my lung puncture is a pneumothorax. So that is how the ward staff saw me: Mrs Pneumothorax. When I first checked in I still thought my primary problems were Addison’s with an uncontrolled chest infection. But hospital processes are not set up to manage more than one thing at a time, and with a lung puncture, everyone from Dr Skinny onwards had seen me as Mrs Pneumothorax. Even Dr Grumpy, the endocrinologist, had overlooked my need for extra steroid medication while the chest drain was put in. Hence my self-medication during surgery.

In which I make my exit

Fast forward to eight days after my check-in. Michelin Man has shrunk down to Paula Radcliffe (just joking). The chest drain came out and I had a nervous 24 hours waiting to see if the lung puncture had healed over.

Then Dr Shy, the chest consultant, appeared. He told me my chest was now clear but he had concerns about my crumbly bones. There was a question mark as to whether I could go diving in future, with the weakness in my ribs. I reminded him that the scan my rheumatologist did for me 12 months previously showed my bone density to be excellent. Then I promised to get the rheumatologist to look at it again, and to confer with the endocrinonologist as to whether I needed Fosomax. We agreed that I now have every incentive to get my daily hydrocortisone dose even lower if I can.


The pharmacist’s party bag makes an encore

Writing this all down in the comfort of my kitchen at home, I am struck by the realisation that the conditions under which my chest drain were put in were not that sterile [5]. Me in my street clothes, and my handbag being passed around among us. That handbag has been a lot of places, and an operating theatre should perhaps not have been part of its itinerary.

This is on my mind, as three days after I stop the antibiotic cover for the chest drain, two days after I get home, I am running a fever again.  So is my daughter, who has a barking cough and – for good measure – a febrile convulsion to go with it.  But there is a chance that my fever is a complication from the surgery.  We go back to the GP for another course of antibiotic. 

Actually I still have some hospital-issue antibiotic from the pharmacist’s party bag that I have secreted home with me. I get straight back on to it as soon as I realise my temperature is up, without even waiting for my GP to confirm an appointment.  He rings back within the hour to say: get that co-amoxiclav down fast.

And that, I hope, is the end of this story.

Ruth

December 2004

Footnotes:

[1] Osteoporosis and osteopenia are significant problems for people with adrenal insufficiency. 17% of respondents reported these conditions (N = 494), compared to only 2% of an age and gender matched control group (N = 327) in the UK Addison’s Disease Self-Help Group’s 2003 survey (unpublished results).

[2] The theoretical ideal replacement dose for adrenal insufficiency is 10 – 12mg hydrocortisone per metre squared.  This is equivalent to 17.5mg – 20mg hydrocortisone per day for the average UK female with adrenal insufficiency, who has a Body Mass Area of just over 1.7.  This compares to an actual reported female average dose of 25mg per day; range 7.5mg – 75mg hydrocortisone per day. (N = 343, 2003 ADSHG survey).  There are significant individual variations in absorption and metabolism and those who are physically active will often require more than the theoretical ideal.  A full day curve is preferable to establish the right regime.

[3] Living with Addison’s disease: an owner’s manual, 2001, published by the UK Addison’s Disease Self-Help Group, available online at www.adshg.org.uk.

[4] Vomiting and/or diarrhoea is the main cause of emergency hospital admission for Addison’s patients, affecting 3 - 6% of Addison’s patients per annum.   Source: unpublished UK Addison’s Disease Self-Help Group email survey 2004 (N = 180).  For an analysis of hospitalisation rates see also Arlt W and Allolio B, Adrenal insufficiency, The Lancet 2003; 361: 1881-93.

[5] Dr Skinny followed British Thoracic Society (BTS) guidelines for the insertion of a chest drain regarding aseptic technique.  The BTS reports a 6% incidence of pleural infection following chest drain insertion in trauma cases such as rib fracture and emphasizes the need for full aseptic technique.  Sources: Thorax 2003; 58 (Suppl II): ii53-ii59, Thorax 53; 58 (Suppl II): ii39-ii52

Self-medication during surgery - The doctor’s view

This case shows how important it is for people with Addison’s to take responsibility for managing their own health – even when surrounded by well-intentioned medical professionals.  If Ruth had not self-medicated for her emergency surgery, it is likely that she would have collapsed during or shortly after the chest drain was being inserted.

This case also shows how important it can be in an emergency for people with Addison’s to take a companion with them to hospital.  Your companion must be informed about Addison’s and able to draw attention to your MedicAlert jewellery.  They should ideally have some documentation with them such as the ADSHG emergency letter, the ADSHG surgical guidelines, or the ADSHG Owner’s Manual. 

Had Ruth been too ill to self-medicate, she would have needed an advocate who could insist that she receive a steroid injection of 100mg hydrocortisone.

Although Ruth did satisfactorily on oral medication for her minor surgery, an intra-muscular injection of 100mg hydrocortisone would have been preferable.  This is because an intra-muscular injection gives a longer-lasting, sustained release of hydrocortisone to provide cover during the surgery and post-surgical recovery period.

Ruth’s story shows that well-educated adrenal patients can keep their own medication while in hospital and take it at their usual times.  But because hospitals have strict procedures to ensure that patients who are too ill to manage their own medication receive it correctly, people with Addison’s will find they need to explain the case for self-medication and have this authorised by a senior consultant.

Ruth does need to be concerned about the potential for damage to her bones from taking too much steroid medication over the longer term.  I would even be a little concerned that she spent three weeks on double dose with flu and a fever.  She would be well advised to have a thorough check of her hydrocortisone levels in a day curve test, as well as a further bone density scan.  Even mildly elevated (excess) doses of hydrocortisone will cause bone loss over time, and osteoporosis occurs in far too many people with Addison’s.

Professor John Wass

The Churchhill Hospital

Oxford

UK

December 2004