Systemic Vasculitis

And then, so I think it was like the Monday I went into the hospital, the scan was on the Tuesday, and it was the Tuesday evening a doctor came to just say that it wasn’t a clot. And then on the Wednesday they’d referred me to respiratory and no-one came to see me until the Friday? And [laughs] the doctor that came, he’s a registrar and he was really lovely. He explained that you know, “We think it might be something called vasculitis? We want to do some bloods tests. We’re not going, we don’t want to say it is that, but we don’t, we want to check that it’s not?”

So again, he was sort of relatively calm, and they also gave me some treatment for an infection in case that was sort of the cause as well. On the CT scan it showed sort of, nodules on the lung, what’s, something called a cavitated lung infarct? Which I think is basically just a gap in your lungs maybe? I’m not entirely sure about that, and granulomas? So initially they thought it was, GPA? Granulomatosis with polyangiitis I think that is? So, they were doing things like ANCA blood tests at that point, which obviously had to be sent away.

So he discharged me, he was like, and it was fine you know, I did a little bit of research on vasculitis and I went on the vasculitis web page and there was a couple of things that I felt could be relevant you know, not just that particular one, that I thought could be related, what I could be experiencing, but I didn’t do too much research at that point because I didn’t want to get too bogged down by it.

And then a couple of weeks later I had a phone call from, well it might’ve been a week or two, I can’t remember, I had a phone call from the registrar and he was like, “Oh, we want you to start some treatment so we’ll need to be, to come down to the ward just to sort of pick up a prescription and things.” At the time I was working in the hospital anyway. So, I was working in A&E with a charity, so I was on shift when I went down. It was one of his colleagues, he was off, so one of his colleagues met me and she took me into a room and she was like, “Okay, this is what we think you have,” so she gave me a document on Takayasu arteritis, which was printed off the vasculitis page, so essentially I’d already read that stuff, but that was the information she gave me, she couldn’t really say much about it herself.

So, I went to A&E and I was admitted immediately. And they said my inflammation markers were so high, they’d never seen anybody coming in with inflammation markers so high. I was then transferred to one of the bigger hospitals and they couldn’t find anything wrong with me. So, I stayed at that bigger hospital but because the beds were in such demand – I mean, they couldn’t see anything wrong with me, so they transferred me back to the local hospital.

In the local hospital I was given, I’m sure, ten different antibiotics, one after another. Nothing worked. I was sent for PET scans, ultrasounds, MRIs. Now, we live in Northern Ireland, I was sent to Dublin, to a big specialist hospital in Dublin for special scans. I was, had a heart echo, I had every sort of - I mean, the National Health Service, it must have cost them a fortune. I had every sort of scan or anything. I had a biopsy on my artery here [points to head] which was really very scary, and during all this time my blood pressure was really dangerously high, and they couldn’t get it down.

So, I was in hospital for six weeks and I was dismissed, and no diagnosis. So, they said the inflammation markers had come down quite a bit, so they were happy to let me go, but they didn’t know what was wrong with me.

Yes, it’s like the baby scan, that’s a, the gel one? And she does from my shoulders, over my head. That, I’ve had three of those.

So, what, are you lying down or?

I’m lying down and she, you know, she puts the gel on me and goes over it and watches her computer. I just see bright colours and everything but she’s watching it. Oh, and the last time I was down, the nurse said to me that that consultant no longer needs to do biopsies, she’s very skilful that she can do without. I don’t know how she does it but, whether it’s all with scans, I don’t, I don’t know.

So, this is like ultrasound scans?

Yes, yes.

Yeah, right, okay. And do you, you said you did have a biopsy?

Yes, I did.

What do you remember of that?

It was two nurses that did it, who were trained to do it. And there again, it was just a, an outpatients’ clinic, and it must have been at least half an hour. It was done down the side of my hairline here, [points to right temple] but afterwards there was nothing, after it healed, there was nothing to see. And I could hear them talking, I could feel them pressing - no pain - but I could feel them pressing as they were trying to get the part they were wanting. It seemed to be very small, what they were trying to get. That, and them discussing it, saying, “That wiggly bit, try and get that wiggly bit,” [laughs] you know? And that’s all I could, you know, I could hear. That, there again, the gel and everything was on, that, you’ve to wipe it all off and get your hair combed [laughs]. But no, it was a nurse, two nurses. One was sort of instructing the other one how to do it. So. That must, whether that confirmed that’s what it was, giant cell arteritis, I don’t, I think so.

So anyway, I came in on the Tuesday, all fully prepped for the biopsy [sighs]. For those who have never had a kidney biopsy it’s, yeah, it’s not, it’s not the most pleasant. Maybe everybody experiences it different, but you lie on your front and you’re still awake and they basically use a really large [laughs] – and stupidly, I kind of looked one time - but it’s like this humongous, big gun [laughs] that goes in your body. And I talk about a gun, because they do, they actually trigger it like a gun, and it takes a bit of your kidney. And they have to do it twice. And, yeah, I did, wasn’t, wasn’t pleasant but survived it, but just it was, horrible feeling actually. Because you have to lie really, really still as well, and you can feel them wiggling around [laughs].

So anyway. So that was on the Tuesday – and then you have to lie still for six hours which is nigh impossible for me to lie still for that long. But my husband was there, so I eventually, I think after about four hours, they could even let your head up a little bit. [laughs] And I was aware that I was quite tender, so on the, I think that was all that happened that day. And then on the Wednesday they - they would come and check on me, and my stats were still going down, so my stats were, were not looking good at all, they continued to deteriorate. And everybody had different ideas, but there was a really lovely consultant who said, “We think it might be something or vasculitis,” and I was like, “What on earth is that?” and she’s like, “Look, I’m not even going to tell you much more about it because there is an expert in the hospital, he is, like, the expert on vasculitis so he will tell you everything.”

But I can’t fault them at the end, when it was bad, the efforts they made to find out what it was, in the last sort of few months before I got diagnosed, once this person had seen the sores on my hand, they referred me to dermatology to look at the rashes, they referred me to ear, nose and throat to biopsy my nose, they referred me to ophthalmology to look at my red eye, I had a CT scan. And then I had to go to the lymphoma doctors because I had enlarged lymph nodes, so they had to rule out lymphoma. So, you can see by the time I actually got diagnosed, I was quite ill. Fortunately, I didn’t have lymphoma, they thought it was all connected to the problem. So, they really did, once they got going, they really did work hard to get an answer.

I was kept in the ICU for about four days and four nights, monitored all the time, they, they’d basically given me drugs to slow my heart down and make me rest, until they could find out what was wrong.

After the four days when it wasn’t so critical, I was moved to a private ward, again there was more scans, more tests, and the, the key presentations I had, was this breathing difficulty with the asthma, it was pains in my back and across my neck, I had lumps on my arms and a rash on my legs, and I’d had these polyps up my nose.

Anyway, this went on for about two or three weeks with all the various different tests and, where I’d been away travelling, they had to check that I hadn’t picked up anything from abroad, I’d travelled to a number of unusual countries. They then started to do biopsies on the small lumps on my arms and give me more scans.

And I suppose I was very fortunate, at one point I had to go to a scan…

…I had a junior doctor come with me, and she was in the ambulance with me, we got stuck in traffic, when we got to [teaching hospital] there was a hold up with one of the machines wasn’t working and we had to work, wait for an hour or so, and then she had to take me back, so she spent the morning with me. And consequently, she really looked into my file. And I think where she was fairly, newly out of college or university, she put two and two together at last and said, “I think this lady’s got Churg-Strauss.” Immediately I said to her, “How do I spell that?” because I knew that my mum would want to know, and she said, “I don’t know,” she said, “It was something we did but I think that’s what the issue is.”

And so then, within the hour, they then got consultants from the relevant department, the lupus [unit at my other large teaching hospital], came over, had a look at all the symptoms and said, “Right, we need more bloods,” et cetera, et cetera, and they said, “Yes, it’s Churg-Strauss,” and then from then on, because they’d identified it, because the biopsy confirmed what it was, they could then start treatment.

But eventually I woke up, in the early hours of one morning in December 2018 and I was really fighting for breath. I live on my own, so it was quite panicky, and I called the GP emergency line, they came to see me, and they immediately called an ambulance, and I was blue lighted into the local hospital, and then from there, the acute assessment unit there diagnosed kidney failure.

And the main diagnostic point that they spotted was that they could smell a kind of urine smell on my breath, which is a sure sign of kidney failure caused by the vasculitis as I was about to find out.

I was then transferred by ambulance to the regional hospital, in [large city], which is the specialist renal unit, or kidney unit, for the region. And over the course of that 48 hours, I went straight into intensive care because my lungs were in fact quite waterlogged, they thought they might have to put me on a ventilator. In the event, they didn’t, which I was very thankful for, I just needed high, high flow oxygen.

I received blood transfusions and a treatment called plasmapheresis, which is where the plasma in the blood is replaced, and that’s to get down the level of vasculitis antibodies in the blood, so that it’s not active, and isn’t attacking other organs.

So, over the course of my stay in intensive care and then on the kidney ward, which was a total of about five weeks altogether, I was diagnosed as having ANCA vasculitis with MPO antibodies, which is also known as microscopic polyangiitis as I’ve come to learn, and that had by the sta-, by the time I got to hospital, that had attacked, both kidneys? Pretty much entirely destroyed one of them and left the other with very little function. So, in kidney terms my, my estimated GFR at that point, which is a measure of kidney function, was nine, and it’s never gone above that since. And it had also caused some scarring, or fibrosis in the lungs, particularly the right lung.