Sarah's now been home from Stanmore for a fortnight, having had her operation a little over a month ago. Getting her home was always the prime objective, but I don't think any of us realised how tough this period was going to be.
A quick walk with the physio.
Daniel from No. 1 catches up with Sarah at the end of her first short walk to the end of the road and back.
Her back has been fine since the very early stages, and she has settled very well to wearing the brace for all but ten minutes of every day. She's even managed to learn to live with the wretched ventilator pump and face mask! But the pain in her left foot has continued to grow, even though it has subsided in the rest of her leg. Increasingly she has found that the painkillers she was given at Stanmore do not cope with her discomfort, particularly at night. As a result, she hasn't been sleeping at all well. Also, she hasn't been getting much exercise so she hasn't been burning off energy, meaning that even her previously poor appetite has dwindled away and her legs have become stiffer with lack of use. A downward spiral.
The crisis came on Sunday night, when it became apparent that her pain was causing enormous distress and could not be allowed to continue. We had Calpol, Paracetemol and Dihydrocodeine, but weren't sure if they could be given together or at what intervals. In any case, whatever we gave her bought her only limited respite. Calling out a GP would not have helped since any doctor other than her regular one wouldn't know anything about her long-term condition or her the specifics of her current needs. So we gave her Calpol and TLC and waited for dawn. Very distressing for Sarah, but also for Anne who is sharing a bed with her at this difficult time and who is with her all day every day.
On Monday everything started to slot into place. The visiting nurse suggested that Sarah might need specialised painkillers that would handle nerve pain, and our excellent GP made a house call and prescribed them. Unfortunately, they take up to a fortnight to kick in! In the afternoon we went to St. Thomas' to try to sort out better arrangements for night-time ventilation, with quite a lot of success. The mask that she was using was too big and she was given the right size one to take home. The complex velcro strapping that Anne has to wrestle with every night was replaced with a simpler arrangement.
Sarah was also given a 'nasal pillow' set-up to try. This still uses the 'Nippy' ventilator, but instead of a mask Sarah has two jets of air directed into her nostrils from a head set that goes over the top of her head. This means that she can turn over during the night and doesn't have to lie the whole time on her back. Also, on checking the machine settings, it was discovered that one of them had been set too high, which might explain why she has found it difficult to tolerate. All in all a very good visit. So good in fact that in the evening we went down to the Bluewater shopping complex, which is VERY wheelchair-friendly, to give her her first, brief, post-Stanmore shopping trip. She even downed a cheeseburger before we left!
And on Tuesday we dashed to see Dr. Issler, the truly excellent paediatrician who has handled Sarah's case since she was born. We had no appointment but sneaked in to see her at 9.00am and she gave us an hour of her time - there was a huge queue when we left! She confirmed that there is no permanent nerve damage and that the prognosis is good. She also prescribed yet another painkiller that will tide us over until the one that handles the nerve pain starts to take effect. Anne has now got a fair idea of which one to give Sarah, why and when. When we left the clinic we also collected a bed frame that raises the bedding from Sarah's foot.
By the time I got home on Tuesday evening, Sarah was feeling much better and had even begun to do some school work. In the evening we gave her painkillers even though her pain was at a fairly low level - she deserves the luxury of zero pain for a while. The side effect of these analgesics is drowsiness, but since she is already so tired through the lack of quality sleep this is hardly a problem.
So, if we can keep the pain right down she should be able to get more exercise, which will loosen up her tight tendons and muscles and make walking easier. It'll also mean that she should be able to consume more calories, which in turn should mean an improved appetite.
This morning (Wednesday) Anne is going to treat herself to an early-morning swim at the local leisure centre and I'm going to 'babysit' - well, I'm going to work at home, which I always find more productive when I've got a specific bit of work to do.
We're clawing our way back towards normality, but we've got a long way to go. But now we know that the light at the end of the tunnel ISN'T that fast-oncoming train!
Sunday 26th March - very early!
It seems like a lifetime since Sarah's operation, but it's only six weeks ago. Her back has been fine since quite early on, but her leg pains are dragging on and show little sign of ebbing away. At times she is weeping in agony at the concentration of pain in the toes of her left foot and she has even said that she wishes she hadn't had the operation. Serious stuff.
The painkillers (she was taking FOUR different types) seemed to be having little effect on the pain but were leaving her drowsy and unable to concentrate on the school work that she needs to catch up on. So, on Friday we took her back to see the orthopaedic surgeon on an emergency appointment. I don't know what we thought he could say or do, but we thought he needed to know what was going on.
He checked the x-rays again and confirmed again that the cause of the pain was due to nerve irritation caused by the epidural and not to the 'metalwork' in her back. He also confirmed that Sarah's recovery from this pain could take a little while, and that she should not let it prevent her exercising her leg. Easier said than done, unfortunately for Sarah, but I'm sure that he's right. Equally unfortunately, he handled her foot fairly roughly, causing her to yelp with pain and dissolve into tears.
He also agreed that it was pointless to be taking such massive doses of painkillers, particularly if they were having no effect. So, we immediately stopped giving her the two more powerful ones, and whilst she has still been in pain, at least it hasn't worsened, so that was a bit of a result! We've now got some liquid paracetemol, which seems to be a little more effective than the painkillers we've ditched. She's still taking the one that deals specifically with nerve pain, which takes quite a while (several weeks, by the look of it) to get going.
Trying to re-introduce her to normal life, Anne took to her to school briefly on two days last week, to catch up on Art and Drama. And yesterday (Saturday) we took her back to her recorder ensemble class at Blackheath Conservatoire for the first time for nearly two months, which cheered her up considerably. And we'll take her to Bluewater again tomorrow, as shopping seems a great way to take her mind off her discomfort, particularly since she's still got loads of holiday money to spend!
So, her pain is moving further down her leg, but seems to be intensifying as it goes. Painkillers don't work very well, so we've cut out most of them. She awakes in agony each morning, and sometimes in the middle of the night as well. But the prognosis remains good, her back is straight and she's three inches taller. It's just heart-rending to watch (and hear) her suffering - she just has such limited physical and emotional reserves left to draw on. But she's fighting back.
We're getting there, but none of us foresaw that it would be like this.
Monday 3rd April
Since my last update, Sarah has spent the best part of a week in the local hospital, with the medics trying to come up with the right cocktail of drugs to ease the continuing pain in her left foot. Luckily, she was able to get a room with a second bed for a carer, so Anne moved in with her again.
I collected them both on Saturday morning, in the expectation that Sarah's pain would still be so bad that we'd have to go back there on Sunday night. However, things went so well that they won't return there until Monday evening, and that is expected to be for the one night only.
The new drugs that she was given at the end of last week now seem to be taking effect, leading to a substantial reduction in pain over the past two days. Additionally, whereas her left foot was previously extremely cold to the touch (if she'd let you touch it!), on Thursday last the heel suddenly warmed up overnight, which the experts all declared with relief was 'significant'. It seems therefore that the recovery of the nerve is continuing. We have absolutely no idea how much longer it will take for the pain to vanish without the need for painkillers.
But, as they say, one door closes and another opens. The 'opening door' is the question of her weight. At the peak towards the end of last year she reached five stone three pounds, but this fell below five stone by Christmas, probably due to her deteriorating condition. On admission to hospital last week she weighed in at only four stone five pounds (27.7kg, I believe), which is cause for enormous concern. We are therefore only a whisker away from her being tube fed, but have bought a four week respite by promising to have a major push for weight gain by conventional means. It's a worry, to put it mildly.
But, to return to the good news, she's brightening visibly as the pain is driven down by the medication. She went to school briefly this afternoon to catch up on some IT work, and was warmly greeted by the Head and Deputy Head, which was nice. She's still in a wheelchair when she goes out, because her walking range is so limited, but now that the pain is going she should be able to progressively go further. We're not rushing this, though. She won't return to school until after Easter, and unless she now makes rapid progress even that is a little questionable
Chatting together one day last week, we worked out that, during March 2000, Sarah had visited seven different hospitals, yes, SEVEN! That sounds like a record to me!
Our thanks to all of you for taking an interest in Sarah's progress. I can always tell that it's time for another update when the telephone enquiries start to mount again!
Saturday 20th May
Sarah's been getting steadily better over the past seven weeks since the last update, and her progress has accelerated further in the past fortnight. We're progressively reducing her medication and abandoning the equipment that we were given when she first came home, and now she's only using the wheelchair and the ventilator. Having said that, she's using the wheelchair less as well!
Unless there's a dramatic increase in her bodyweight she'll still need to be tube-fed this summer, but that's been postponed until after her GCSE exams. These have now started and she's determined to sit them all. Fortunately, we have so much supporting documentation about her case from so many sources that we've been able to ensure that she can take her exams under the best possible conditions.
For all of her written papers she has been given the use of a separate (quiet) room, where she can take a break for a short rest if she likes and can have more time if she needs it. The support that we've had from the local Education Authority, Health Authority, school, doctor, paediatrician, surgeon, etc. has been incredible - well worth the taxes that we pay!
The surgeon said that Sarah could have up to half an hour a day out of her brace, but only around the house and only if she was extra-careful. So, since we were worried that when she's told in August (we hope!) that she can stop wearing it altogether she'll be too nervous to do so, we've encouraged her to take advantage of this relaxation. And if her eating has improved recently it probably has something to do with the shock she had when she took the brace off and saw herself in a mirror.
On Friday evening I took her to see the new Tate Modern museum of modern art by the Thames at Southwark, and persuaded her to leave the wheelchair at home. She managed to walk and stand for an hour and a half before we had to head for the car, which I think was a fantastic achievement. This still left us with 80% of the exhibits still to see, so we're planning to return.
She's still using the ventilator at night (although she's having an experimental break from it tonight). She's been told that she won't need it in the long run, but that a couple more sleep studies will be necessary before she can stop using it, provided that the results are satisfactory of course. We've cut out one of the nerve painkillers altogether and we're cutting back sharply on the paracetemol as well. We're also finding that she doesn't need to have an afternoon sleep each day, and this must be related to cutting back on the drugs.
And in spite of the drug reduction the pain in her toe continues to die away. In fact, this evening she said that quite a lot of the time she feels no pain at all, and that when she does it's a sudden shooting pain that doesn't last. It's also nice to see that the colour of her left foot is now more or less normal, so her circulation must be improving alongside the gradual increase in the exercise that she's taking.
Sarah on holiday in Dartmouth, August 2000
The news is good all round. Firstly, and most importantly, the pain in her foot has now almost vanished. From time to time she still gets shooting pains, but most of the time she has no pain at all. And that, of course, has meant that she has been able to progressively reduce the painkillers that she has been taking, and now takes just one tablet once a day. In fact, we believe that she could probably get rid of that one as well, but she is sensibly waiting until the next appointment with the paediatrician (Dr. Issler) before she does so. We still have the wheelchair, but she seldom uses it, unless we're out for the day and there's the risk that she won't be able to find somewhere for her to sit and rest from time to time.
Earlier in the summer the dentist at Great Ormond Street Hospital said that she could remove the braces (retainers) on her teeth during the day, wearing them just at night. That was a small but important step back towards normality. Better still, last week we returned to Stanmore for the six months checkup, and, as we hoped, Mr. Morley said that she could also discard her back brace. Now that *is* progress!
Sarah and Mr. Morley, August 17th, 2000 Sarah and Dad, August 24th, 2000 (GCSE results day!)
So, that leaves the matters of her low bodyweight and her breathing difficulties. She's still using the 'Nippy' ventilator at night, and this was made more tolerable a couple of months ago by a humidifier supplied by St. Thomas' Hospital. This has stopped one of the longer term problems, i.e. her airways becoming dry at night due to the dry air rushing in and out. We found out about humidifiers through another group of new friends - the members of the international e-mail list for people with Sarah's muscle condition. It's not what you know, it's *who* you know! She's reluctant to experiment with not using the ventilator at night since, on a couple of nights way back when she didn't use it, she woke with a headache, feeling sick. However, during the last week or so, she's been waking reasonably early, switching off the machine and then having her two hour 'lie-in'. And, it seems, her breathing has improved to the extent that she doesn't feel too bad after a couple of hours unsupported sleep. We live in hope that she'll be able to get rid of it entirely in the foreseeable future - might still be quite a while, though.
As far as her weight is concerned, there's good news. From the disastrous 27.7kg of early April she's moved on to approaching 33kg! The bad news is that she's only been able to achieve this through tube-feeding. This started nearly two months ago, and so progress has been slow, but we're all delighted to see her gaining weight. She now has yet another machine by her bed - this one holds a litre bottle of concentrated nourishment and vitamins, the contents of which are dripped down the tube into her stomach at 125ml an hour over eight hours while she sleeps. In the morning she, predictably, has no appetite, but by lunchtime she feels peckish and at some point between then and early evening generally eats, and enjoys (!), a reasonably substantial meal. It's great to see her, for more or less the first time in her life, eat the occasional meal with genuine pleasure and without us feeling the need to hassle her to eat more. Dr. Issler is looking for a weight of between 35kg and 40kg, before the tube comes out. It will stay in for another month or so, perhaps more, so she'll, unfortunately, have to go back to school with it stuck to her face, but she's a tough individual and will take that in her stride.
And today we had great news. You'll recall that, in the middle of her recovery, she had to contend with her GCSE exams. She was really determined and managed to sit them all, and this clearly paid off, as we heard today that she had passed them all, achieving grades in the important A* to C range for all of them! We couldn't be more happy for her, as this success is hers and hers alone. She received enormous support from her school, from teachers and friends, and this provided the launch pad for her own determination and abilities.
Sarah collecting her school prize on the last day before the summer holidays
And where from here? Well, she goes back to school in a fortnight, to enter the sixth form and study (probably) English, History, History of Art and Music at 'A' level. She's still quite frail physically, but she's rock-hard mentally, and we're sure she's got the right outlook to enjoy the next two years at school.
What a relief!
She's now back at school,
studying for the
Levels she was interested in (English, History, History of Art and
plus a fifth that the school urged her to do - General Studies. Her
continues to rise encouragingly (now approaching 35kg) and she's still
growing taller (she grew 2mm during August alone!) I think she'll stick
with the tube-feeding for a while yet, as she's grown used to it and
how beneficial it is to her general health. Let's hope that we can then
wean her off the night-time ventilation as well.
Thank you all for listening. Our
your greetings, and our very best wishes to you all.