Charlie
Charlie has granulomatosis with polyangiitis (GPA), an ANCA associated vasculitis affecting his lungs, ears, nose, windpipe and pituitary gland. He breathes with the aid of a tracheostomy tube in his neck, sealing it with his finger when he wants to talk.
Charlie is a statistician for large-scale research projects including clinical trials. He lives with his wife and their baby daughter. Ethnic background: White British.
More about me...
In 2018, Charlie started getting sinus infections and nose bleeds. As these became more severe, the tubes connecting his nose to his ears became blocked, leading to complete hearing loss. He had swelling all over his body, with headaches and night sweats, and was in such pain that he needed morphine. By this point Charlie says he was incredibly unwell and reliant on his wife.
Charlie recalls that, on one A&E (accident and emergency) visit, a doctor suspected vasculitis. Within days rheumatology took over his care. Charlie was diagnosed with granulomatosis with polyangiitis (GPA), a type of ANCA associated vasculitis, and given high dose steroids and rituximab to suppress his immune system.
Although he quickly felt better and his hearing eventually came back, a few months later Charlie experienced new and rare GPA symptoms. The first – excessive thirst and frequent need to urinate – was a sign that his pituitary gland was affected. The second – difficulty breathing and always sounding out of breath – was a result of inflammation in his windpipe. Both needed immediate action, and Charlie was given cyclophosphamide (chemotherapy) and throat surgery.
As repeated surgeries weren’t doing enough to maintain Charlie’s airway, he had an urgent operation to make a hole in his neck just below his voice box. This meant he could breathe through a tracheostomy tube but has to use his finger to seal the end of the tube every time he speaks. Although Charlie’s breathing improved, his voice became increasingly unreliable. Hand gestures and an electronic larynx (a device which vibrates against the voice box) were difficult to use and his wife ended up speaking for him. At this point his rheumatology team decided to give him a second round of both rituximab and cyclophosphamide. Fortunately, this combination was effective, and Charlie partly regained his voice.
In August 2020 Charlie was relieved when his throat biopsy came back clear, and all the infusions, operations and tablets appeared to have brought the vasculitis under control. He describes himself as learning to live with the condition, the side effects of treatment, and uncertainty about the future while reducing his medication. He says he will have further major open throat surgery, with the aim of removing the tracheostomy at some point.
Charlie says the loss of independence has been the most challenging aspect of vasculitis and that counselling and support with mental health have been important in adjusting to his new life. He has appreciated flexibility from his employer and access to a disability mentoring scheme at work.
While grateful that he was diagnosed relatively quickly, Charlie is disappointed that the health system for vasculitis care is so disjointed. He says coordination of his care is the thing he and his wife have to think about every single day. Having a new home and baby, Charlie hopes that, in future, vasculitis will have less impact on their day-to-day life, and that his wife’s forthcoming child’s storybook about vasculitis will help other families understand the condition.
When numerous appointments were “going nowhere,” an advanced nurse practitioner pushed for Charlie’s symptoms to be taken seriously and urgently.
When numerous appointments were “going nowhere,” an advanced nurse practitioner pushed for Charlie’s symptoms to be taken seriously and urgently.
I think one example was, for me, at early stages of having different symptoms that were seeming quite acute and overt and more than that you would expect for someone with an infection. And I went into my GP surgery and was seen by a advanced nurse and it was a really important point for me because she looked, observed these symptoms and thought that something really clearly seemed wrong here, in that the repeated appointments - numerous, numerous appointments at the GP surgery with different doctors - were just going nowhere and, really, clearly something else had to be done. So.
She made a very in-depth evaluation of all the symptoms over about 45 minutes and then after that, she then, while I was in the room, was calling up the hospital and the specific department and was then explaining these things through and saying, “I really think that there’s something funny going on here, please could you, kind of let, could you allow it, kind of bring him in this afternoon, and to be to take all the paperwork that I’ve done and then kind of see what you think about this.”
Steroids had an “amazing” impact on Charlie’s vasculitis symptoms. A biologic drug (rituximab) had a more gradual effect.
Steroids had an “amazing” impact on Charlie’s vasculitis symptoms. A biologic drug (rituximab) had a more gradual effect.
And I was still incredibly ill at that point and was reliant on being carried, kind of needing support from others, and not very able, and deaf at that point. No hearing at all. And immediately, I was meeting kind of doctors in rheumatology, and a few days later, was started on high-dose steroids and rituximab.
And it’s amazing with the steroids, that within two days I went from feeling incredibly ill to feeling much, much better. It was, it was amazing, the impact of it. Suddenly within two days, night sweats went down, swelling went down, loads of pain went down, and we started on the rituximab that continued on for a number of weeks. And that was all incredibly helpful, and they were such a supportive team. And the hearing loss and, and the deafness continued for some time, but then eventually at some point, the inflamed parts around my adenoids at the back of the throat kind of shrunk and then was able to clear the fluid and my hearing came back, which was a wonderful experience.
And at that point, things were looking quite good. I’d had kind of solid treatment, I’d been reducing my steroids, my symptoms were improving, I was able to go for long walks and be able to be outside a lot.
Charlie’s vasculitis affected his airway. He had throat surgeries and a tracheostomy to help him breathe.
Charlie’s vasculitis affected his airway. He had throat surgeries and a tracheostomy to help him breathe.
And it didn’t feel quite right, so I went back to the doctor’s, and we did some tests, and then found that there were kind of two new sides of vasculitis that were - quote - more rare, that I’d started picking up for some reason, but hadn’t started before. One of them was a rare one which is inflammation of the pituitary gland, and what that impact has is kind of inducing diabetes insipidus, which means that you get very, very thirsty and you need to go to the bathroom much more. So that one was identified and that’s quite a high-risk one because the damage on the brain can be quite substantial from that.
And then the second one was that, after we did different tests and did a lot of work with ENT, we found that I’d had something called a subglottic stenosis, which is essentially inflammation, the thickening of tissue in the windpipe, just below the larynx. And what that meant was that that was getting much larger and stopping my ability to breathe.
So, with these, both these things, we suddenly had to make and kind of take quite serious action at that point. So, I had to immediately be put on cyclophosphamide and have these treatments every, every two weeks and in addition to that, I had to have numerous surgeries on my throat to be able to maintain the airway. And then this continued for a number of months, but it got to the stage that - because the surgeries were so ineffective and would only keep the airways open for three to four weeks - at that point it got, it kind of got so urgent that I ended up having a tracheostomy, as you can see now. And that was a very major surgery and I had to be in hospital for a long time at that point.
But even though it was incredibly challenging and required feeding tubes and numerous other kind of very difficult things within the hospital, as you kind of don’t realise how many things you lose from kind of having surgical work done on your throat, after that I was able to breathe easily for the first time in about six months, which was, in itself, an amazing thing. Which, which was really good.
After chemotherapy for vasculitis, Charlie was like “a Victorian ghost.” He feels it was hard on his wife.
After chemotherapy for vasculitis, Charlie was like “a Victorian ghost.” He feels it was hard on his wife.
I think some of it is really just the challenge of seeing someone so ill, I think. I think its examples were, like, I mean, yeah, these are horrendous ones where we would be in lockdown or something else going on, I would leave the house to visit the hospital, I would have my cyclophosphamide treatment for the whole day, it was really, really long. Then I’d come back, and I would look like a Victorian ghost, I just looked so ill and unwell and a million miles away from the person that left the house in the morning. And then my wife had to experience that and see that, and to then try to minimise the problems for me with all of that and I think it’s incredibly challenging to see someone from a treatment that will help you in the long-term, to affect someone in such an awful way, to turn them from being themselves into something that isn’t themselves. And I think for family, that’s incredibly difficult to see.
The effects of vasculitis and its treatment has, at times, had a profound impact on Charlie’s identity.
The effects of vasculitis and its treatment has, at times, had a profound impact on Charlie’s identity.
I think for me, the main, I think the main thing that I felt with illness was the loss of independence, really, and I think the increased reliance on others, either with my body not being able to function in certain ways or be very unpredictable or being reliant on others for communication, for hearing, for speaking, and having to think so much about my own breathing and all the treatments that have gone with vasculitis. I think, definitely, the impact on yourself and your identity can be very profound, I think, for those periods. And to lose such independence at a young age is very difficult, and I think that could be very hard for a lot of people to, to deal with.
Vasculitis affected Charlie’s windpipe and voice. He often depended on his wife to speak for him.
Vasculitis affected Charlie’s windpipe and voice. He often depended on his wife to speak for him.
And then after that, I continued to have, my breathing was better, but I was starting to get a loss of my voice. What that meant was that on some days I would have a voice, on other days I would have no voice, I would often have to mouth or lipread and my wife would tend to have to speak for me. And at some points, in January, I ended up really just being mute and not able to speak at all.
And I was, just after that point, my daughter was, was born and during that whole period in hospital and everything around that, I couldn’t speak. And my wife had to speak for me, even through all those points, or I had to try to gesture.
And this was a, kind of an effect of this subglottic stenosis, and stopping any air kind of going above the tracheostomy.
Charlie needs sick leave for vasculitis treatment. It helps that his organisation is “compassionate.”
Charlie needs sick leave for vasculitis treatment. It helps that his organisation is “compassionate.”
Or, if other people were to think about it in different ways, I suppose you would think about something like a CV and, and, and what you put on a CV, and the longer the time that you’re off on sick leave and unable to do the things that, that, that you would like to do in work, that CV would become more and more empty or missing kind of large gaps.
So, you kind of feel like, though it’s help, helping your health at the moment with this treatment, that in the long-term you are almost being penalised for not being able to maintain what you need on your CV to be able to kind of continue on in the work that you would like to do.
So, I think that is hard for anyone with a condition, and I think the most important thing that, that can go along with that is some levels of compassion or care from people in the organisation or managers, to at least understand those worries and fears, I think, and at least acknowledge them. It’s not necessarily the case, I don’t think, in any real world that people can truly deal with those and truly, completely take away any of these worries for people because that’s just work, that’s, that is life. But I think for people within the organisation to actually be compassionate and to care and to listen and to understand that those worries are there, I think at least halfway allows someone to know that people understand the challenges that they’re going through. Not necessarily that they can change them, but that they understand that they’re there.
It’s possible Charlie will lose his voice permanently as a result of vasculitis. In case this happens, he is recording his voice so his daughter will always know how it sounds.
It’s possible Charlie will lose his voice permanently as a result of vasculitis. In case this happens, he is recording his voice so his daughter will always know how it sounds.
But it is incredibly challenging. I would say for those people that have completely lost their voice and got to the point where you cannot make any sound, there really aren’t many options out there. And even now, when we’re thinking about the future, for me, it’s very uncertain about whether I will maintain this voice or completely lose it altogether permanently, for my, for the rest of my life because we’re still uncertain about whether I can actually have procedures on my throat, or whether they’re not, they’re not viable.
And if they’re not, we’ve had to think a lot about things like voice banking and the ability to save thousands of words and once kind of having to say those words, to be able to work with companies to be able to develop a system in which you could type and then it can come up with your original voice, to be able to say these words. And people, like, different people in the US, have been known to be able to use these devices and to be able to communicate in this way, but I think it’s still quite a new thing. But it would always have limits again on having to hand-type out what you want to say and then it, for it to come up.
Apart from that, I’ll be honest, we can’t think of that many other options that, that, that could be done, really, for me, at the point if I completely lose my voice. So, I think it’s quite a, yeah, at the moment we’re, there’s a lot of uncertainty around that and I think that is definitely quite difficult. So, we’ve even tried to do things like increasing doing videos with my daughter or increasing different recordings, or just, just maybe even, just things for our own family, of saving the voice. In, just in case, within a few years I lose it permanently and my daughter never knows the sound of my voice when, when she’s older.
Charlie’s wife is writing a children’s story book about vasculitis. As a family they would have found this helpful after he was diagnosed.
Charlie’s wife is writing a children’s story book about vasculitis. As a family they would have found this helpful after he was diagnosed.
We’ve been thinking about it a lot here. I mean, what my wife’s currently doing is a perfect example, she’s just recently written a book on vasculitis, about vasculitis, and this book is a, what you would probably call a children’s story book. It is designed for a family audience to start those early discussions or conversations about vasculitis and maybe their grandparent or someone in the family having vasculitis and beginning to slowly bring different people in the family into the fold to talk about it, but in a way that isn’t necessarily very scary and very clinical.
And part of the, so the way that the story, the story book works is it is a story about a family and someone within the family having the condition and being written from a child’s perspective, but there is lots of information within it about what different doctors are called, what different tests need to be done for vasculitis, what different feelings and symptoms and things that can happen to people with it and how it feels, and what might be their day to day, and. She then went on to forums and then asked people, “If you were to read a book, what would you find most helpful for people to do or to know to find, to be helpful for you?” and she added little bits on that about a daughter finding out about how she could help her dad and what little things she could do, and... It was a really, I mean, she’s a great writer but it was really her ideas about how to present something in that way that I think was so novel, to create it in a way in which it wasn’t intimidating, it wasn’t scary, but allowed some information to come out that gave someone that may know nothing about it to get quite a good grasp on it.
And I think the other part about it that most people don’t really want to acknowledge is that the majority of us don’t, don’t, we don’t really know that much about things in that area generally, I mean, we might have done biology at school or some other bits, but not really at the stage where we’d really understand these things. So.
There are many, many different adults and other people that don’t really understand what this diagnosis is and what’s happening, but you’re kind of quite embarrassed to say, especially when you have doctors that are very informed and very, kind of speaking with this very kind of clever language. So the other part of the book - that was kind of quite subtle but a big part of it - is allowing adults to feel like they can be informed and learn about things in, in, in more of a gradual way, but in a way that doesn’t feel like it’s belittled or that it’s embarrassing, because it’s them reading this with their child or with children and that kind of connection between the two, to basically inform a lot of different people around this.
Charlie found vasculitis support group websites helpful in some ways and unhelpful in others. He’d prefer more “relatable” and less “scary” content.
Charlie found vasculitis support group websites helpful in some ways and unhelpful in others. He’d prefer more “relatable” and less “scary” content.
Parts or examples within that were things like, a lot of the stories that you would find on these websites would be very much based around people that you would really call success stories. But quite extreme in terms of success, so someone who would have a condition and get vasculitis, they would end up in ICU, they would have all these treatments, then afterwards they would become a marathon runner and they would do runs in seven, eight different countries. Or you have someone else that once they had finished their treatment, they had started their own business and now they were running something in the UK. Or someone else that was doing, doing something kind of, almost something that you couldn’t really relate with because it was so extreme in terms of having a condition and achieving something so kind of far out there. And with those stories not really acknowledging all the bits in-between, but also not really finding something that was that relatable.
And then the other part I think as well that I think I found quite difficult was that, within these newsletters and within the information for these charities, there would often be an obituaries section. And I will never forget, when I was first diagnosed, I received my first newsletter, I was going through, finding bits of information. And then on the obituaries section there was someone who had been diagnosed the same age as me, she seemed to have the same background as me, and started with the same condition as me, and the same, the same symptoms that had come up and then she had died ten years later. And they said she had just been, she had been battling vasculitis for a long time and kind of, yeah, that she’d passed away from it. And I think, [smiles] I think things like that are pretty horrific to read, I mean, at points where you trying to get a newsletter that, I don’t know, you find you’re in a community or you connect with different things and there were parts like that in it that personally, I just found horrific to see.
And then the other part that was then really difficult was all these research kind of parts that they add in to the newsletters and other bits, that are so impersonal and clinical and talk about very scary complications and things within it that, yes, for a research standpoint where you can be objective, it’s so important to know these things, but the way it was presented in these newsletters was, I think, in a way that was, firstly, it was on a very clinical standpoint so not that easy to read from a non-clinical point of view and secondly would then include things that can be quite hard to read if you’re seeing written down specific risks or different things that can happen to people.
So, I think I got a lot of mixed messages, and although there was a lot of information that was helpful within it, I think there was a lot of things that were unhelpful as well.
Charlie finds his vasculitis healthcare “incredibly disjointed.” Poor communication in a “mad system” leaves him “almost a hundred per cent responsible for my own care.”
Charlie finds his vasculitis healthcare “incredibly disjointed.” Poor communication in a “mad system” leaves him “almost a hundred per cent responsible for my own care.”
So, you have this absolutely kind of mad system, really, that does not work, where you have this group that you’d call rheumatology, that are essentially the generals and the ones commanding things, and then under them there would be specific specialists in different organs. For example, you’d have the renal units for the kidney, you’d have respiratory teams for the lungs, you’d have ENT for the ear, nose and throat, and you would, could then end up like me, a tracheostomy, having other specialist teams.
And, essentially, the way that it works is that it doesn’t work [smiles]. It doesn’t work at all, so you have rheumatology generally do the referrals and they ask you to see the other people, but the other people don’t have that much knowledge about vasculitis. But if they do, my experience is that none of them communicate at all, and not very well. And if it is, it’s done by a short letter that doesn’t provide any real detail or granularity. They never contact each other via telephone, they never contact each other face to face, they have no idea what the other person, has never read into what the other doctor’s decisions have been.
Rheumatology, when I ask them questions at all about specific organs, they often shrug it off or will say, “Oh, we, we’re not specialists in that, we don’t know,” but rheumatology need to be the commanders that are able to manage these other parts. And then when I go to specialists and we discuss things, they firstly don’t even call it rheumatology, they often get the words wrong and they say, “Oh, what’s your immunologist said?” or, “What’s this person said?” and they use funny language. They often have never read the notes on what the latest updates are from rheumatology about my activity in my vasculitis, and then they will do their procedure that they are familiar with, and then not really give that much feedback on the overall part of that, and then basically just send a letter back to rheumatology saying what has been done.
So, the system at the moment, where I live, is incredibly disjointed. And I would say that what that has led to is really just me being almost a hundred per cent responsible for my own care. I have to manage every doctor, I have to manage every specialty, I have had to find very - contact details that were definitely not in the private domain - in the public domain, sorry. For example, I now have access and had to get different ways to get access to private emails of PAs, for many different phone numbers for different parts of the departments, just to be able to contact the different people that are managing my care, and to be able to manage what’s needing to be done.
As well as using their clinical knowledge, Charlie would encourage doctors to ask people about the wider impacts of vasculitis on their lives.
As well as using their clinical knowledge, Charlie would encourage doctors to ask people about the wider impacts of vasculitis on their lives.
So, I don’t know if at any point you’ve had people who have discussed the wider impact on your life and asked you about that, even if there’s nothing they could, they felt they could do about it, has that formed any part of their, what they’re thinking about when they’re making decisions?
My experience is it depends who, it depends who the doctor is. It depends who that person is who is making the decision. I think that I felt that there are definitely a number of senior consultants that very much have taken a lot of things from understanding lifestyle and impacts and a lot of things that are going on, and really factoring that into their decision-making process but also discussing that at appointments. And those are the ones, and those are the doctors that stick with you, those are the doctors that you feel grateful to have met and feel the connection to and feel supported by, and you feel like you’re never going to forget them.
And then there is a very good chunk, and I would say – I’ll be honest - it would always be the less-senior doctors that haven’t developed that side. And I would definitely say there’s a whole rank of seniority. And, apart from a few that were incredibly good who were quite junior, I would say that the lower down my experience was of seniority and experience, the feeling that it was less, kind of something that was, I would say a focus? But also, I would say was encouraged by more senior people for them to think about during these consultations.
I think it’s quite a wide thing, in my experience, of different doctors within the local hospital that I’ve had here, and I think that there is a lot of pressure on younger, the younger doctors to very much have a very good procedural and clinical knowledge, and to be able to gain a lot of experience, both medicinally but also from kind of on a surgical standpoint.
But, from what I’ve seen and heard, there seems to be very little guidance, support and training around more of the personal side and more of the side of thinking about a patient more as the whole rather than as a clinical interest.