Usually with autoimmunity you find that it runs in families. Although there isn’t a history as such in my family, I do have a young family member who was diagnosed with HSP (Henoch-Schönlein purpura, how is that for a mouth full?!) not too long before I was diagnosed with AIH. I asked my auntie and uncle if they would like to share their journey with you guys on what they went through with Kenny, as these illnesses affect everyone so differently, also to see through a parents eyes, so, here goes, Aunty Al, over to you!
One mum’s experience of their 4year old boy developing HSP nephritis
Hello, not ever written a blog style thing before. But my wonderful niece Maddison thought some guest pieces about other autoimmune illness experiences could go well on this brilliant and moving blog of hers, so here goes (for the record, I think Maddison is a total hero).
I’m trying to think what sort of thing I would have liked to have read 16 months ago when we were in the middle of it all. Probably mainly about how other parents have been to the edge with their kids, but come through to solid ground again. I remember clearly thinking ‘this is how it goes for some people, you get here, then it just keeps going and it doesn’t get better, no miracle happens and you never wake up from the nightmare.’ A bit like looking down into a tunnel. So I don’t really think I can write anything else without throwing something out there towards those parents who were forced down to the end of that tunnel. But it wasn’t us last year and I can’t speak for them. My son Kenny looks and acts like any other healthy 5 year old kid now and yes, I am grateful every day and still find it nearly impossible to believe (even at the same time as I am yelling at him to stop moaning / fighting with his sister / brush his teeth…)
Phase 1: HSP, ‘not mild’
Completely healthy kid until the day after his 4th birthday party when he got a rash on his lower legs that looked a bit meningitis-y; plus he was sick a few times. Took him down to the GP and she advised us to get him down to the a&e that night as she suspected HSP. From what others have said we were lucky to get a GP who recognised it straight away as the illness is rare (14 cases per 100,000 school age children in USA – rarediseases.org). The first few weeks of HSP are typically (but not always) intense. He had continuous and excruciating abdominal pain, not holding anything down – moaning and cramping up at regular intervals all day and all night. Other symptoms on top of the abdominal pain and rash (which quickly spreads all over legs and arms into nearly total cover) is swollen joints and joint pain. It’s caused by the immune system mistakenly attacking the body’s own small blood vessels, causing them to burst or become inflamed. Kenny barely walked for about two months in total and eventually he had to re-learn.
For some kids this is ‘all’ they get, at which point it is ‘only’ HSP (Henoch Schonlein Purpura, btw) and tends towards being a childhood illness where subsequent flares, if there are any, become less and less severe until it goes away. Adults can be sufferers too tho it is less common and tends, unfortunately, to be more chronic, with flares often thought to be linked to stress and other triggers (not always known). Last year the info on the NHS website said that it was a ‘mild illness’ that clears up within 4-6 wks. They seem to have updated that a bit as it now says that it is ‘not usually serious’. In any case, it is not mild. We were in and out of the (- thankfully still open Lewisham -) hospital multiple times even in the early stages. From all the facebook groups that I am on, one thing is clear – there is really no saying how long it will last. Yes, it could be a month. Or, it could be years. Or come, go away for 10 years and come back again. That’s because it is a systemic (whole body) autoimmune illness and the way that I understand it, once your system has malfunctioned like that, there is a much higher likelihood of it doing it again to some degree. It is incredibly variable from one sufferer to another.
Phase 2: HSP nephritis
In about 50% of cases in kids the immune system also starts attacking the kidneys, causing inflammation and possible scarring of the tissues. That’s what happened with our Ken, at which point it becomes HSP nephritis, so then we were in and out of the hospital monitoring his kidney function (blood and urine tests). For some kids, even with kidney involvement, all that is required is monitoring and it eventually gets better on its own.
Phase 3: HSP nephrotic nephritis
It is normal with HSP to get random swelling e.g. in the face, balls etc. So when his face started swelling up we put it down to ‘just being HSP’ (we were in between hospital monitoring days). After a few days of it getting progressively worse and him not being able to open his eye that morning (I had taken him to the cinema the day before!!! Carrying him in…), I got the feeling that something was really not right. I spent about an hour googling his symptoms and found a page on infokid.org about nephrotic syndrome so I told my husband Grant I was going to take him down to the a&e again just to be sure. That day ended with Grant accompanying him by ambulance (an experience I am told it is probably best I missed) to intensive care at the Evelina at St Thomas’ and a full blood transfusion amongst a tonne of other things (I got there just after they did). His kidneys were leaking so much protein because of the damage being done to them by his own immune system that all his fluids in his body went wonky and everything was shutting down. This is rare but it does happen. We were transferred to the kidney ward after that first night where we stayed for about 2 ½ weeks.
Phase 4: immune suppression vs chicken pox
On top of all that, and it still gives me a cold rush to think about it, his sister Esther, 2 years younger than him, had got chicken pox a few weeks earlier and by a random meeting, another mum had told me that chicken pox can be lethal with kids that are immune compromised. We had immediately separated the kids for about 5 days and nights (which was weirdly one of the hardest things emotionally I had to do in that period and in fact I would have broken had it not been for Grant insisting we should stick it out) and we didn’t know if he had got it or not. On the plus side this meant that we got our own room in intensive care and on the kidney ward, but the horrific side was pretty horrific. The quickly fitted in biopsy (which I also managed to miss) showed that in order to stand a chance of saving his kidneys they had to go in immediately with extremely high dose prednisolone (3 day IV ‘pulses’) BUT if it turned out he had chicken pox, they may not be able to save him as under those conditions chicken pox can take over the body. Kidneys vs staying alive. A chicken pox looking spot came up, I saw it, they ran tests (involving the virologist expert coming in to work on a Saturday off her own back), 24hrs later they said it WASN’T. By which point they had already decided to run the risk (based on the dates we gave) and start the high dose pred anyway.
Phase 5: pred
And, as Maddison has talked about, prednisolone is a whole other kettle of crap. Kenny was on an extremely high dose for a month (60mg per day for those that know about these things, I understand that the uppermost limit even for adults is around 80mg per day), then on a 6 month taper. He was unrecognisable to look at (moon face, barrel chest, hunched over), in character (fearful rages, hunger) and, something that really affected me even though it doesn’t sound like much, to carry (no longer hanging off me like a limpet but so top heavy I could hardly lift him). Intense times in a whole different way. But bloody hell, the pred did what it was meant to (along with 10 or so other meds, including Tacrolimus which he is only now tapering off) and his kidney function is incredibly now at the high end of normal, down from literally off the scale and almost certain dialysis / transplant. Real effects with real side effects. In our son’s fast-moving and life-threatening case, it seems that we were extremely lucky to have access to these meds.
Impact on Kenny
Still with me?! Kenny in those days was dark eyed, frightened and angry. He would growl when doctors or nurses came into the room and would scream through the daily blood tests, canulas etc. These days he is completely back to his cheery, chatty (most of the time) self and has even come on massively from his straight up phobia of blood tests / needles caused (understandably) by that time, though he still has a phobia of ‘in the body’ – all those grotesque cartoons when brains come out or they go down the throat and all that stuff. You wouldn’t belive how often it comes up! He knows that he ‘has HSP’ and he still has to take 3 meds orally per day so we keep the conversation open about eating well, resting, getting exercise etc and how all his hard work is helping him stay healthy. A trick I learned from Grant to help him with his needle phobia is to try and get him to give his arm to the nurse – it’s about him taking some control over his situation and it not being another traumatic thing forced on him. I’m not sure when or how we will tell him the full extent of what he went through. Maybe when he’s old enough to read this himself! AND, of course, we don’t know whether he will have any further flares and whether his kidneys will hold up or end up going down the chronically damaged route (teenage years are likely to be a particularly risky bit, because of both the changing body and the lifestyle e.g. alcohol etc). I plan to do a bit more reading about how open it is helpful to be with him now that we have put some distance between us and all the horror.
Cross each bridge…
Which leads me onto the impact on me. Of course there was also the impact on Grant, on me and Grant as a couple, on Kenny’s sister Esther (she was young enough for most of it to go over her head, though she sometimes has a chip on her shoulder about not being special I think, she quite often says she’s ill too..), on our family and friends (where would we have been without them) but it’s sort of nice to have the opportunity to talk about the impact on me too a bit.
There is a lot I could say about coping strategies and the emotional peaks and (very deep) troughs, including grief, and anger I didn’t know I had, and coming back from intense emotional vulnerability, not to mention the weird sort of ‘suffering relativism’ to all the other bad sh** around BUT I want to try and angle this to the particular stresses of the autoimmune stuff. The main thing that caused this surreal, extra layer of complexity was never really knowing what we were dealing with. Official info seems to be massively lagging behind the real life experience stuff you pick up (facebook groups are an incredible resource). So it was me who had to come to the realisation on my own of the likelihood of the illness being serious and long-lasting. I came to this realisation fairly early on during one particular very late night obsessively reading stuff online prompted by the deeply scary sight of my boy looking so ill. But it’s those impossible questions that no one can answer – how long is this going to last? Do I have to give up my job? Can someone tell me what the **** I should tell my work? I did leave my job but was lucky that they left the door open for me to come back further down the line on reduced hours. I clearly remember it feeling like everything was dissolving around me – plans for the day, plans for the summer, plans for work… letting everything go completely, including the thought that ‘he’ll be better tomorrow’. Eventually it reduced right down to looking no further than the next minute or max, day, this long period of suspended and concentrated time. It was months before we were able to start rebuilding the holy grail of Normal Routines.
But back to the – more – confusing bit. Autoimmune illness seems to come with this enormous pit of contradiction and lack of clarity. Two long-serving consultants on the same ward had differing views on the use of steroids. No one knows definitively why HSP is triggered, whether it is viral, bacterial or other triggers (it is sometimes but not always preceded by upper respiratory infections, for example). The genetic susceptibility is sometimes mentioned, sometimes omitted or denied (I have seen it referenced in medical papers online, but only as a part of the picture – environmental and other things almost certainly play a part too). Because of this lack of clarity, and frankly when you are desperate, it’s hard to resist getting drawn into endless hours of reading stuff online without necessarily getting any closer to anything helpful. In my view, evidence based medicine (or filtered-down-to-the-person-on-the-street evidence based medicine, anyway) is currently missing a trick on this one, with little to say about the ‘soft’, long-term, joined-up treatment. This therefore leaves the door open for all sorts of pseudo-science: some seems to me to be reductive and even predatory, some is almost certainly (in my view) enormously valuable. But you have nothing to judge it all by except your own instinct. I also don’t think most people can understand medical papers, I certainly can’t. So the result is that you are more likely to tune into the emotional stuff. Because of the impossibility of all of this I have always just settled back at not doing anything crazy or extreme and sticking to the basics: a healthy balanced diet not over reliant on junk, hydration, low stress and rest (I’m happy to call him in sick at school for a rest day if I think he needs it, and I’m sure to tell the school that that’s what it’s for as I think they need to learn about it too). I do think there is almost certainly something to be said for things like the Autoimmune Protocol Diet (AIP) but it is hardcore with no guarantees and because of my feeling that we have to be so careful with diets when you are talking about kids (and especially kids with damaged kidneys..), in the end I just try to adopt a few things that sound sensible. I don’t necessarily think that these things are the ‘answer’, or the ‘cause’, but it does seem that for some people they can be a big part of the answer…
One acknowledgement and two little stories to wrap this up:
Acknowledgement: our consultant at the Evelina is an incredible, hard-working NHS doctor. We feel unbelievably lucky to have been under her care, and everyone else who works so hard in our hospitals as well, not least the intensive care consultant and nurses, and everyone at Lewisham children’s a&e.
First story: the mum in the kidney ward who responded so generously to my unmasked fear and told me about her son who had never walked or not eaten through a tube, but who had a great sense of humour and had loads of friends in his class – she told me that with time, everything becomes normal. I suppose that the goalposts get moved but, given half the chance, we adapt, and what else can we really hope for.
Final story: a barrel-chested moon-faced Kenny whacking the sofa like a drum with plastic sticks and dancing with me and Esther… Testament to his spirit and his pride (and probably some chemical buzz..) – good lad our Ken 🙂