Long overdue

Gosh, I hardly know where to begin.  I've been wanting to update you for such a long time, but it's been an extremely difficult and traumatic few months.  Five months.  Gosh, it's five months since I last wrote here.  I can't possibly tell you all that has happened in that time in one post, so I shall just aim to cover the immediate period following my last post.

I started the betamethasone a few days after last posting here, while I was still in hospital so they could watch for any immediate difficulties, and we did a straight swap from 60mg prednisolone to 9mg betamethasone.  Thankfully the swap was uneventful and I was able to go home a day or two later.

It very quickly became apparent that I was absorbing the betamethasone very differently to the prednisolone.  The first noticeable difference was that I was bouncing with energy.  It was a false energy that I did know from previous very, very high doses of pred, but never to that extent.  I easily managed on four hours of sleep a night, and this was immediately after the admission when I knew that my body was actually exhausted and needed rest, but I couldn't.  I was bright and was eager to get on with a big declutter of my flat, starting with the bedroom.  Every day was filled with sorting through everything in my bedroom - in the desk, in the wardrobe, under the bed, in files I hadn't looked in for years.  I shredded years of old diaries, bank statements, old business paperwork; took bags upon bags of things to the tip; donated at least nine black bin bags full of things to the charity shop; cleaned the whole room to within an inch of its life; a friend ended up redecorating part of the room for me; I dismantled furniture and constructed new Ikea furniture; and then I started on the living room.

It was very hard work, but I had the energy so I got on with the decluttering while the energy lasted, because I knew that as the dose of betamethasone was weaned down the energy would begin to falter.  Of course the activity made me sweat, but I sweated excessively, even sitting shredding hundreds of pages of paper a few sheets at a time would soak my clothes through with sweat.  But whilst I was sweating, and sweating, and sweating my body was swelling, and swelling, and swelling with fluid. However much furosemide (diuretic) I took it did nothing to rid me of the excessive oedema, and I was/am on a huge dose of 120mg twice a day.

Then there was the hunger.  Of course, using a lot of energy with activity meant that I needed more energy in the form of food, but not that much more, and my hunger was excessive for the energy I was using.  I tried not to give in because I am already far too overweight and in desperate need of losing some, but it was incredibly difficult.  My hunger was never satisfied.  I could eat what I knew to be a normal-sized meal and I would feel as though I'd had a micro snack.  I could eat what I knew to be a big meal and I would feel as though I'd perhaps had a light spot of lunch.  It was awful to be permanently uncomfortable with hunger yet seeing the weight pile on, which combined with the excessive oedema to change my appearance significantly.

My face, my huge moon face, lost all definition.  My nose shrank into my ever growing cheeks, and my chin and jaw were lost to the depths of my neck.  This look is typical of someone on high dose steroids, and it is rightly named because the face becomes spherical like a full moon.  Yes, I have been moon faced for many years, but never to that extent, never to the extent where it felt like a facial disfigurement.  A whole body disfigurement with the extra weight and fluid too.

The disfigurement continued because my skin couldn't keep up with the rate at which it was needing to expand, and my whole torso, tops of my arms, and tops of my legs have become covered in huge, ugly striae.  These are essentially stretch marks, but they are so much more than stretch marks.  They are deep purple and red lines, some as wide as three or four centimetres, almost looking like huge lacerations snaking their way up my body and down the backs of my arms.  The skin is so thin that in places I can clearly see the fine veins below.  Sometimes they split and I bleed.  They happen quickly too, so quickly that I feel them happening - the fibres of my skin ripping apart, forever damaged, and now that I've lost a little of the weight again, the skin hangs limp, wrinkled, flaccid, separate from the tissue below so that I can pull it with my fingers like stretched chewing gum and just about see my fingers through the two layers of skin pressed together.



This is not me, but it may as well be.  This is what my torso looks like, except that the striae on the front of my body go all the way up to my breasts.  It is at least a disfigurement that I can mostly hide under clothes, except those at the tops of my arms and the top of my chest that often peak out from under my T-shirts.  Of course, there is no hiding them from a medic when I am examined, and although I warn them, and they are used to seeing such things, I still see the shock flicker across their face before professionalism hides it again.

I am told that over time the colour will fade, but I know that the skin will not, cannot, repair itself, and although they may become silvery, they will always be visible.

The plan was to try to reduce the betamethasone dose fairly quickly so that I could try to get to a more normal prednisolone-equivalent dose.  I was nervous about doing this because of so many failures of dose reduction with pred in the past, and the resultant life-threatening asthma attacks, but given the rapid destruction of my body I was willing to try.  In some ways this was made easier by the fact that each betamethasone tablet was a much smaller dose than each prednisolone tablet, so my lungs kind of didn't notice the reduction so acutely (although it also meant that to begin with I was taking eighteen betamethasone tablets each day!).

I thought that the quicker reduction in betamethasone dose would mean an equally rapid reduction in side effects, but in actuality, the only one that did subside as quickly was the false energy.  That was actually something of a relief, because while it was good to be living more in the same time zone as everyone else around me, I could feel that my body was, in reality, completely exhausted.  But one side effect that actually continued to increase was severe weakness in my thigh muscles in particular, but other muscles too.  I quickly became unable to stand from a chair without pushing off something, and if I ended up on the floor (as is the wont of someone like myself with POTS) then I was basically stuck until someone could help me up or I could somehow clamber onto my footstool and from there shuffle onto the sofa, where I would still have to brace myself for the huge effort and several attempts to get to standing again.

I went to my GP in the end, not really because I expected him to be able to do anything, but simply because the whole combination of all the symptoms, and their rapid progression, was getting to me.  I came away with an official diagnosis of Cushing's Syndrome. Of course, because I have been on such high doses of steroids for such a long time, and because I am aware of their many, many side effects, I have been known about Cushing's Syndrome for a lot of years.  I have known that I have exhibited many signs and features of it at various times to varying degrees over those years, but there was still something rather shocking about receiving the diagnosis.  One thing I have had particular difficulty with is the prognosis as fifty percent of people with untreated Cushing's Syndrome die within five years of diagnosis.  My CS cannot be treated as the only treatment is to remove the cause, which in my case would be to remove the steroids.  Remove the steroids I die either of an asthma attack or adrenal crisis, as my body is now dependent on the prescribed steroids because it can no longer produce any of its own.  I can only hope that I am in the other fifty percent.

Whilst all of this was going on with the explosion of betamethasone side effects and the additional diagnosis of Cushing's Syndrome, another problem was developing.  There was a supply 'issue' with the betamethasone at manufacturing level.  I had been given enough in my initial prescription to keep me going for quite a while, but before too long I needed another prescription.  My local pharmacy was able to honour the first three hundred tablets on that prescription, but that wasn't going to last me all that long, even though I was reducing the dose and therefore eking out the medication.  I managed to get in touch with my consultant's secretary and Dr H wrote a hospital prescription for me that he hoped would tide me over until my local pharmacy was able to source the rest of the meds they owed me.  The hospital out-patient pharmacy had some, but not enough, and they had to do some internal negotiations to borrow from the in-patient pharmacy so that prescription could be fulfilled.  They normally wouldn't be able to do this, but as the problem extended into community pharmacies as well then they made an exception.  However, this was not a problem that was going to be resolved in time for the next prescription, and before too long I found myself with only enough tablets to last about six more days and then I'd be without any steroids at all.  This wasn't an option.

I had been keeping J, my Complex Respiratory Disease Specialist Nurse, informed all the way through as the problem arose and progressed.  He liaised with my consultant, and the three of us ended up having a discussion about it in clinic, by which time I was about three days away from prescription crisis point.  Dr H had to come up with a new plan.

One thing had firmly been established - that I clearly hadn't been absorbing or utilising the prednisolone as I ought to have been, and that I clearly was absorbing or utilising the betamethasone very differently.  This added credence to  the hypothesis that not all steroids are equal, and I needed a different one.

Dr H decided, as I hoped he would, that it would be foolish to go back to a reliance on only prednisolone as it obviously wasn't working satisfactorily.  However, we also knew from the side effects that prednisolone had given me over the years that I had been absorbing at least some of it.  So Dr H started me back on 40mg prednisolone alongside an injection of 40mg IM triamcinolone every three weeks, with instructions to reduce the pred relatively quickly to 25mg.  I wasn't able to reduce it quite as quickly as Dr H had wanted me to, in part because my lungs did notice the reduction, though I did eventually get the dose down to 25mg, and then subsequently to 10mg.  Ideally he would like me to get to 5mg pred, but so many things have prevented further reduction for now.

The other reason I was unable to reduce the prednisolone down to 25mg as quickly as Dr H wanted me to was because I ruptured my Achilles tendon.  Most people get this injury through contact sports, or extreme adventures, or a vicious football tackle, or some other 'exciting' activity.  I did it by slowly walking from my living room to my bedroom.  I did nothing unusual.  I didn't trip.  I didn't fall.  I didn't stand on anything, though I did go back to the 'scene of the crime' to see if I'd trodden on something.  No, I simply stepped forward as normal and then crunch!  Sudden extreme pain, followed surprisingly quickly by relatively little pain, but I couldn't walk properly and it felt 'all wrong.'  Another trip to the GP and I came away with an 'urgent 24 hour referral' to an orthopod at the hospital.  Only this 'urgent 24 hour referral' took almost two weeks to come through.

It transpired that the betamethasone/Cushing's Syndrome has also weakened my tendons and ligaments, and the orthopod took literally two seconds to confirm the diagnosis of a spontaneously ruptured Achilles tendon.  Obviously with my lungs in the state that they are he was extremely reluctant to operate, so it is being treated conservatively - with a moon boot.  For the first two months I had a hard moon boot (like that shown in the link) to wear through the day, and a softer, lighter one to wear at night.  Since my check up in mid-October I have been able to stop wearing the night time boot, but I still have to wear the heavy, cumbersome one through the day when I'm up and about.  Only now I have a matching pair of moon boots, because the stress put on the left leg by the heavy, cumbersome moon boot on the right leg has caused the Achilles in the left leg to tear.  Thankfully it isn't a full rupture, but for the foreseeable future I have a pair of moon boots to go with my moon face and alien appearance of my torso.

This is far, far from all that has happened since I last posted, but it is enough for now.  I will return very soon with a continuation of the update, but as you have now read some of the traumas of the past few months you may be able to appreciate a little of why I have felt too overwhelmed to write about it.  Instead I have been living it and trying to muddle my way through.

Acceptance

It was a long and difficult bank holiday weekend with no answers to all my questions, no conversations with anyone with authority to give any suggestion of answers, and a lot of time to think and fret. I was able to talk about it a little with my mum and step-dad who'd come down from Edinburgh to see me, but visiting times can sometimes pass too quickly, especially when you're trying to make space for serious discussion without being miserable all the time. It can be a difficult balance. We did talk a bit, though, and I cried a bit, and Mum gave me a hug, and neither of us came up with any answers because we're the people with the questions, not the answers. Then all too soon Sunday afternoon arrived, Mum and J went back home, and I had an extra day of weekend to get through before I had any chance of asking the questions to folk with the answers.

Tuesday morning and an email from J in response to the email I'd sent him on Friday. It said that he could understand my frustration and he'd speak with Dr H. I thanked him and waited. I didn't see J today when I thought perhaps I may have done, but he was likely very busy with the curtailed week. I presume, though, that at some point he did have that conversation with Dr H that he said he would, but I'm not sure if it was before Dr H came on his ward round.

At first there was no mention of any of last week's discussions. It was all about what progress I'd made since Friday, how I was feeling this morning, what my peak flows were doing, and planning to get the aminophylline infusion down. All important, but not what was at the forefront of my mind. That came at the end when he asked if I had any questions. I thought he might be a little exasperated when I again rose the subject of the steroids, but he wasn't, and although there was still a lot of that professional detachment from Friday, there was a little more emotional engagement too.

One of the reasons Dr H had given for not giving me triamcinolone was that there is no clear dose equivalent of it to the 60mg of prednisolone I take. A friend had then sent me a link to a web page with a conversion table for different steroid medications, including triamcinolone and prednisolone, which of course flew in the face of what Dr H had said. I asked him about this today and he said that there is a dosage equivalence for the two medications in tablet form, but not converting from oral prednisolone to intramuscular (IM) triamcinolone, and with that he really doesn't know what dose he would give me. That made more sense to me than him just not knowing.

We talked some more about betamethasone, and I put to him my concerns about the lack of any evidence for its efficacy. I have actually found one paper online about its use in severe refractory asthma that's unresponsive/minimally responsive to prednisolone, but it was on a tiny cohort of only twelve patients. Dr H didn't know about it. He said he hadn't known any research had been done on it; it had just been a thought he'd had once and decided to give it a try with a few patients. I have to say that it did surprise me somewhat that he wasn't aware of the paper, but at least he said he'd look it up. So then I went on to say that there had been more articles online about triamcinolone, and more anecdotal evidence of its efficacy, including J's introduction to me of a patient who's been on it for seven months with good effect. I also said that I've spoken to friends who've tried it, some with positive results, and again it's the lack of any kind of evidence of betamethasone being effective that bothers me. Dr H reiterated that in theory there really shouldn't be any difference in their efficacy because a steroid is a steroid is a steroid, but he did then go on to tell me of one man he treated, who had been on high dose prednisolone for a long time with minimal positive effect whilst having a number of the negative side effects (all much like me). After four weeks of being on betamethasone he had piled on the weight, but he was breathing better, had more energy, and was quickly able to reduce the betamethasone dose to levels equivalent to much lower doses of prednisolone. It's only one anecdote of one patient, but it is at least some evidence.

I would like to be able to say that we came to a compromise, but we didn't. What actually happened was that I relented. Or came to accept the situation. Or something in between.

Perhaps it is acceptance. I have accepted trying betamethasone. I am still hugely disappointed that I'm not going to have the opportunity at this time to try triamcinolone, but to be fair to Dr H he did say today that 'It's not an either or,' and that if we can get my overall steroid dose down to more reasonable levels with the betamethasone then we could perhaps give triamcinolone a go (the advantage then of the triamcinolone being that it would mean I could take fewer tablets overall as the steroids would be given as an injection). I guess that, to a degree, I've accepted that betamethasone is my only option ... and all the stress and upset and fretting over the long weekend has exhausted me into a place of thinking that if betamethasone is my only option then at least it is an option, and there haven't been any of those for a very long time. I will try it.

We are hoping that we can get the aminophylline infusion down tomorrow.  The dose has been reduced over the past several days so hopefully stopping the infusion and restarting the oral theophylline will be uneventful, but it's always a slightly anxious time as I've had many disasterous attempts at this in the past. Given that Dr H and I have come to this 'agreement' regarding the betamethasone, I said I needed to know what the plan now is. I can't now be sitting on maybes and waiting for some undetermined start date; I need to know when it will be started. Thinking about it, Dr H didn't actually give an exact day, but said that we'd get the aminophylline down and they'd start it while I'm still in hospital because they will need to keep an eye on me. To me this suggests it'll be sometime in the next couple of days, but I kind of feel like I need to make sure it does actually happen.

Dr H has warned me that my body will probably miss the prednisolone in a number of ways. He didn't say how, but said that I might not feel great. It's not exactly a physical addiction to the prednisolone, but it's something akin to it. I don't know how much of 'not feeling great' and 'withdrawal' from the prednisolone is going to be overridden by the betamethasone going into my system, but then I don't know if there'll just be a straight swap, dose for dose, or a gradual decrease of prednisolone alongside a gradual increase of betamethasone. I suppose these are things to ask and find out about over the next day or two.

Steroids

Dr H came this morning as expected, but didn't broach the subject of triamcinolone and a change in steroid medication. We discussed the immediate aspects of my treatment and the fact that I'd had a very bad night last night, and then he asked if I had any questions. Well yes, I did. The big question hanging over me was about trying triamcinolone as we'd discussed earlier in the week before he'd gone away. As I said yesterday, I had thought Dr H had got some measure of where I'm up to with my asthma in terms of physical and emotional stamina, that he'd understood, and maybe he has, but something had changed by this morning. Perhaps it was his time away doing whatever he's been doing for the rest of the week; or perhaps he applied professional emotional detachment (not always a good thing, in my opinion); or perhaps he's just a man being a man who doesn't want/like to 'do' emotions. Maybe it was something else. Whatever it was, though, was harder and less approachable. Less forthcoming. Almost less understanding.

I realise now that I'd slipped up and had allowed myself to let a smidgen of hope squeeze through the cracks. Maybe it was helped along by the positive vibes I had got from J, and having been introduced to someone for whom triamcinolone has been beneficial. I think I'd told myself that that introduction wouldn't have happened if it wasn't at least likely that I'd be allowed to try it, and that J saying, 'He's game if I am,' was almost confirmation that it was practically decided that I'd be given this opportunity.

I am a fool.

I had been led to believe that J and Dr H would be having a conversation about it all, and then would follow a conversation in which I was included too. Perhaps the first of these conversations happened, but I'm not convinced.

This morning, when I asked Dr H about triamcinolone, he basically said no. Now it's not quite as straightforward as that, and I know he's not dismissing everything I said to him on Monday, but I feel almost bereft. The reasoning behind this retraction is that there is no direct equivalent of dose between prednisolone (my current steroid) and triamcinolone, which makes the transition complicated. Dr H knows what dose of triamcinolone he gives to patients on a much lower maintenance dose of prednisolone, e.g. 15-20mg, but not for the dose that I take - 50-60mg. He said that they'd have to give me so much that he's not sure it's feasible/possible. I understand that. I can appreciate that. The logical part of my mind can see where he's coming from, but the emotional part is screaming that surely this must have crossed his mind before and if so why mention it in the first place and plant those first seeds of hope?! It is unfair of him to have done that! I'm cross! I'm fuming!

I'm broken.

But I said before that the no is not a straightforward no. It appears to be a no for triamcinolone, which is shattering, but he said that they might yet try a different oral steroid, beclomethasone. I think I mentioned it briefly in my previous post. It's used a lot in asthma in its inhaled form, but not so much as tablets. In fact I can find nothing about its use in tablet form in the treatment of asthma when hunting on Google. Neither have I heard the same anecdotal evidence for its efficacy in asthma (as tablets, not inhaler) when prednisolone has failed as I did for triamcinolone. This isn't to say that it won't work or doesn't have the same chance of working as triamcinolone, but that it doesn't seem as likely to me because the evidence isn't as forthcoming.

Perhaps my upset over the apparent no to triamcinolone is out of proportion given that beclomethasone is being presented as an alternative, but the lack of evidence or anecdotes of helpfulness does bother me. I know there were never any guarantees that triamcinolone would work for me, but it sounded possible. Beclomethasone feels much more like a stab in the dark, with a vague chance that changing the steroid drug might help but with no/little past experience on which to base any solid hope. Given the option I will try it, because after all I have absolutely nothing else to try, but I don't have anywhere near the same level of hopefulness for a positive result. I was such a fool to allow myself to begin hoping for triamcinolone and the possibility of the positivity that seemed to be being presented to me. I should know better by now than to allow myself to begin hoping when it comes to 'new' asthma treatments for me.

I emailed J this afternoon. I told him what Dr H had said and how the triamcinolone has been retracted as a possibility. I told him of my upset and confusion. I told him I need his help so I can understand what the hell is happening with these apparent opportunities to try to get some life back. I heard nothing back. One of the ward nurses said J had popped up to say goodbye before he went home but he'd seen I was asleep and hadn't wanted to disturb me. On one level I appreciate that, but on another I'm cross because I thought it would have been apparent from my email that I very much needed to discuss the steroids and all the questions I raised.

Now it's the weekend and neither J nor Dr H will be in. It is also a bank holiday weekend so I suspect neither of them will be in until Tuesday. That's a long time to wait for answers that have potential to impact on my life so enormously. Maybe you don't see it that way. Maybe Dr H doesn't understand it as much as I thought he did. But for me it feels like the difference between life and none life. I want to live, but not like this. This is not living.