Fran’s Story
I’m a fifty-two-year-old woman diagnosed with the allergic form of bronchopulmonary aspergillosis (APBA) in 2016 after three or four years of symptoms and signs; then developing semi-invasive chronic pulmonary aspergillosis (CPA) in 2017.
What I’d like to get across to you with my story is that over the last five years, I have experienced such kindness and innovation in the face of shrinking NHS resources, that although this condition is very scary at times, I have had the best care and the best coordinated care – which are two different things. At the very end of this story I’ll conclude by some comments on what the system needs to deliver for people with aspergillosis based on a wider range of patient experiences that I happen to know about through the Aspergillosis Trust.
A quick history of me
From the age of four, I was a very atopic wheezy child and missed a lot of school. My asthma was scary, my eczema was limiting, and my hayfever overwhelming. Then I hit puberty and was transformed. I went on to enjoy 25 years of robust good health.
Cut to 2011, and I started getting regular bouts of what my GP said was bronchitis, which I put down to being worn out and stressed. I had a baby, a four-year-old, and my dad who I was very close to, had just died. Then in December 2013 after a night in A&E an hour’s drive away, with my mum who was having the latest emergency associated with the risky behaviour of early stage dementia, I felt absolutely terrible with an unstoppable foul-tasting productive cough and fever. The following morning my husband took our children, aged 3.5 and 7 at the time, to school. The next time he saw me I was in ICU at Homerton Hospital and I was gravely ill with septic shock and pneumonia [which I’d left untreated for 24 hours], and we were told the next six hours would determine if I lived or died.
I lived and oddly was completely well for nearly two years until I had the idea that I was going to develop a new career cultivating an apple orchard that had been left to run wild for thirty years [midlife crisis – don’t ask]. Tangled up leaves thick with furry mould and fungus brushed my face repeatedly… then I wondered why I had worsening shortness of breath over a three-month period. Because she was concerned we weren’t getting to the bottom of this, my super GP referred me for a chest x-ray in the gap between Christmas and New Year. I decided to go on New Year’s Eve because it would be quiet. Indeed it was, and I was home 20 minutes later, only to receive a call almost at once. I had to have a CT scan as soon as possible but services would be shut due to New Year, so I had to wait for a while. In the interim, my mind ran riot and I read up about every single lung condition on the internet. Aspergillosis sounded vastly better than many of the other conditions I terrified myself thinking about. When I was found to have some specks on my lungs that needed onward referral to a respiratory outpatient clinic, I told the doctors about the mouldy orchard and raised aspergillosis as a possibility.
Then two years of hopes raised, hopes dashed
There was early optimism – the kindly locum respiratory registrar phoned me on his last day at the Homerton, while I was doing some last-minute Easter shopping. He said – “yes it’s aspergillosis, I’m prescribing a few weeks of steroids, and by the time we review you in clinic, I’d expect those specks on your lung to be gone”. The sense of my good fortune was reinforced by a GP at my practice who said “Well that must be a relief not to have anything really nasty”. I felt very lucky and trusted the opinion I’d been given on how this would be sorted out.
Eight weeks later I was admitted to hospital for two nights with sepsis and chest infection. After the hospital admission my very kind local respiratory consultant fitted me in at the end of a massively long afternoon clinic and said “I don’t really know what to do with you next but I guess we could try antifungal tablets.”
I got a referral to the Royal Brompton Hospital as I felt I needed a doctor with experience and expertise in this condition. It was really reassuring to be assessed by exactly such a person and even better to hear I could immediately come off antifungals, and to be told that although I did have some bronchiectasis, it was at such an early stage it could be reversible, As a result I went on holiday thinking I was very lucky indeed and this was going to cause me few problems in life.
I came back from holiday, to a two page, very considered and helpful report from the expert consultant but written later by hand – ‘PSEUDOMONAS – VERY SURPRISING!!! There began two years of repeated heart-sinks. Over the next 10 or so months I did have various treatments for pseudomonas – culminating in a ten-day admission to the Brompton for IV antibiotics. Being in such a specialist centre, I had a lot of tests and got the blow that the speck had become a nodule, and I had the chronic version of the illness. My memory of ward round was hearing that this is not something that happens to every patient but of 220 patients in clinic with the allergic type of aspergillosis, I was the third person this has happened to. A couple of years on, I get the sense through other patient stories and some stats I’ve seen, that it may be more common for this transition to happen. Having a rare illness felt quite bad enough. When it made the switch, on a 1-in-70 chance, to the worse version of the rare illness, that was a blow that left me initially very down. I was reassured by what I’d been told, that the antifungal tablets I’d come home with would help my new problem. I was also on a high because the pseudomonas treatment worked like a dream and I left hospital feeling like I had new lungs, and ran two 10k races in the next few weeks, coming in just behind the elite runners in one of them.
Meanwhile a right old brew of life plus medical trouble was afoot, so even as I smashed my 10k times, I was blissfully unaware of what was to come next. Although I’d left hospital prescribed with antifungal tablets, my feet swelled up a couple of weeks in, and I was advised to come off the tablets and be reassessed in clinic at an appointment that was more than two months away. If I had not been preoccupied, I would have queried this length of time with no new care plan and no medication, and asked about alternatives. But as it was, I was deep in a time of huge stress and grief to do with my mother and the end stages of her dementia. Dementia is a very cruel illness and as an only child, I had no siblings to help. My mum had actually gone at this point in the sense that dementia had gradually been taking her, so I had lost my mum. Grief does unusual things to people. For me, I totally unprecedentedly ignored the onset of a chest infection and the start of a urine infection. I had literally lost my usual very strong will to live.
I got home from a stay at my mum’s therefore, and ended up unconscious on the bathroom floor with a broken nose and cheek bone. I had sepsis which had caused my blood pressure to drop. Lovely London Ambulance Service paramedics pulled me out from under the bathroom sink and I got admitted to the Homerton. This resulted in an eight-day admission and eight days of worsening news about my prognosis. But actually it was a turning point that I came to view as very fortunate in an odd sort of way.
Because there was a hiatus in my treatment plan, and I’d not really got started on antifungal tablets before I stopped them, the nodule had grown at what I was later told was an ‘alarming rate’. I got moved to a respiratory ward where the person in the next bed died a few minutes after I was installed in mine. I got told erroneously it turned out, and by a doctor who was very caring to me but unfamiliar with my condition, that nothing could stop my cavities from growing. My best friend from school turned up and wept all over my bed and told me I looked like the Elephant Man because of my bashed-up face. I was also negotiating when I felt well enough with Occupational Therapy services in another part of the country about the logistics of two carers hoisting my mum, back in her deteriorating dementia world. My husband brought our children in to see me and I had to pretend to be cheery. It was rock bottom.
However this emergency exposed the growth of a cavity in my lung and the seriousness of my situation, some weeks ahead of a routine clinic appointment. The admission started the superb communication and joint working of my case between the Homerton and the Brompton that has characterised my care ever since. No one else I’m aware of with this condition has been as lucky as me in this quality and level of communication, much of which includes me, between specialist aspergillosis team and local respiratory team.
It also saw me taking control of my own care. I was assessed for IV antifungals and was the first Homerton OPAT (Outpatient Parenteral Antibiotic Team) patient to be lined up for home administration of my IVs, starting with the next round. I was taught how to mix my meds, prime a line, and set my drip up for a 90-minute infusion. I was able to base myself in the kitchen at home once my line was in, and the freedom this gave me was empowering (not a word I use lightly). My mum went into the final days of life, and as misfortune would have it, it seemed very likely she would die during my IV week. I talked to the nurse and consultants about this, and they came up with a solution that quite honestly overwhelmed me – I could take three days of IV supplies with me, leaving right away, because they trusted me to do the self-administration. And the consultant rang her opposite number in my mum’s locality to say I might turn up if I got in a mess and could they be prepared to help me. I coped, and on the second day, my mum died and I was with her. This would not have been possible without the can-do attitude of the Homerton OPAT.
This can-do attitude was apparent as I continued my routine of six weeks off IVs, one week on. During 2018, an infection called Mycobacterium abscessus gradually took hold of me. The Homerton team had long exchanges of emails with my consultant at the Brompton to see if the treatment could be done as an outpatient there. Reluctantly having read what it was going to consist of, we all agreed that I had to be admitted to the Brompton. One of the many things that I think is great about the Brompton is that a lot of thought goes into making what I guess are complex treatment decisions concerning people with aspergillosis. Another consultant there also knows me a little, as an inpatient. I was hugely impressed and reassured to hear he had made his team, who were looking after the inpatients the month I was admitted, sit down and recreate the timeline of my illness from my records, so they knew about me. By now I had an issue with believing anything positive, which is unusual for me, and I felt fearful of where I was going to end up with the combination of semi invasive aspergillosis and Mycobacterium abscessus. I felt I had had my hopes raised the previous year, repeatedly, only to become alarmingly ill with aspergillosis and then to find out I had Mycobacterium abscessus as well, which sounds as though it can be difficult to treat. I asked to see the psychologist attached to the team, and while I was admitted we did some work on this issue of trust between doctor and patient. I was blasted with three different IV antibiotics for two weeks, and the outcome was that, looking at my CT scan, my consultant said the outcome was better than he’d hoped for and the improvement dramatic.
In the intervening period since I have became unwell, to the vastly better place I am for the moment, my specialist consultant has been unfailingly kind and above all responsive to me. This is so different to what I think the popular conception of a patient / consultant relationship can be. One example of this responsiveness has been that from the time I came into clinic following my emergency hospital admission and the revelation about the speed at which one of the cavities in my lung was growing, until very recently when he semi-retired, my consultant asked me to communicate directly with him via email. I also used this to talk to my equally responsive local OPAT consultants so we all had a dialogue which I was part of. That really is empowering.
My aspergillosis is currently stable and I don’t have to have my IVs every 7th week. The freedom is amazing. I’m carefully monitored. To go back to the hopes raised / hopes dashed, this is part of the developing relationship of trust between doctor, the rest of the MDT, and patient. When I was an inpatient at the Brompton and very low, I asked the other consultant who knows me whether I should be downcast or optimistic. He said I was someone who for some time, the concern had been that they couldn’t really get a handle on my situation. He now thought I was about to turn a corner and could foresee a time when I could do without IV antifungals and just be taking the tablets. I happened to re-read my diary of that admission the other day, and this is what has in fact happened. I think with all my doctors now I have frank conversations about what the odds are for certain things and I ask more questions. Alongside that I now feel very solidly included in decision making, respected, and treated as an individual.
In conclusion
I was lucky because of the communication around, and co-ordination of, my care. I’d like to move out of London but daren’t disrupt my perfect quartet of specialist hospital, local OPAT and hospital, GP practice, and endlessly helpful pharmacist round the corner. I figure this is a bigger prize than living somewhere with cleaner air. Co-ordination is what all aspergillosis patients should have, and especially when we are too unwell to do it for ourselves. Even I, who have a dream team, still spend substantial time on liaison. I get the best care, and I feel anger and outrage reading other people’s experiences in our patient group, because this should not be a postcode lottery, but it is. Communication and advice from the expert to the less experienced local respiratory team is essential. I’m again unusual in having that working as well as it does. Primary Care is where even bigger variation lies. For some people with aspergillosis, any sense of a team working to support them is non-existent.
Medical teams are only part of our lives. The Aspergillosis Trust and also the virtual patient groups established and moderated by the National Aspergillosis Centre in Manchester, are huge sources of support. We are not just patients – I was the mum of young children frantically juggling a demented old parent, somehow being a together sort of professional, and doing a bit to try and support other people who have aspergillosis. All the other people I know virtually with this illness have the same rich backstories but I’m not sure they are always heard or responded to.
I include here
OPAT TEACHING VIDEOS FOR SELF-ADMINISTRATION OF IV ANTIBIOTICS
the link to a film made with the Homerton OPAT which covers their work with me and has me talking about aspergillosis. *Vanity disclaimer: Unfortunately I had fallen over running two days before and broken my jaw and cut my chin. Also I wore what I thought was the most perfect blue shirt for ‘on camera’ only to discover it was the same colour as the hospital curtains, which has the effect of making me look like a disembodied head.
