Heather's Ankylosis

Last updated 8 February 2008

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11 January 2001: Over recent months, it was starting to become obvious that our daughter Heather appeared to be incapable of opening her mouth properly. We mentioned it to her dentist who told us that it was merely a malocclusion and referred us to the local orthodontist. This led to an appointment after about 6 weeks. The orthodontist took one comparatively brief look at her and tentatively diagnosed Ankylosis of the Temporomandibular Joint, a deformity of the jaw joint - he had been involved in a survey of TMJ disorder sufferers including ankylosis when he was at Guys Hospital in London. Within a matter of days, we were on our way to the Maxillofacial Unit at Queen Victoria Hospital in East Grinstead to see a specialist.

Some x-rays were taken, somewhat blurred as Heather was, at under three years old, understandably reluctant to lie still on a metal table in a very forbidding looking room. A second set of x-rays were taken, the results of which were not totally conclusive, but the specialist did not think that immediate action would be necessary. A forthcoming CT scan is to be taken to get a more accurate picture of what might be happening.

We were at first concerned that it might have been damage incurred when she fell down a concrete step and bashed her face, although the specialist thinks this unlikely as once her teeth had stopped bleeding after ten minutes, she was in no other discomfort. In this case the condition is more likely to be genetic.

Currently, we have been told that as she is gaining weight normally and talks well for her age that any operation will be deferred until she is old enough to cooperate properly in physiotherapy, although action may well be required sooner if further problems develop. Essentially, the jaw joint has to be rebuilt, possibly using a bone graft from the ribs depending on the extent of the deformity.

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22 January 2001: CT scan today. We were woken up at about twenty past six in the morning by Heather vomiting over her bed - not a good start! No follow ups, so we assume it was brought on by coughing as she has a cold. No breakfast for her because she is to have a general anaesthetic. We arrive at Dolphin Ward in the new wing at Medway Maritime Hospital more or less on time, and the sedation goes without a hitch, apart from a mild reaction to Amyltop, a mild anaesthetic cream they use to numb the skin where they insert the needle to top up the anaesthetic if needed.

11am and wheeled around to the CT unit - lift her from the trolley onto the machine's bed. The eyes then open "I want a wee!!!" Having sorted this little problem, we try to lay her back down afterwards so that she will doze off. This is unsuccessful until we try laying a blanket over her to make her think she is in bed, that is. Fortunately this trick works! I have to remain in the room, wearing a stylish lead lined coat and collar, in case she wakes up and panics because there is nobody there. No worries, though, as she remains snoring throughout. The Radiologist checks that the scan is okay, gives us the nod and we can leave. We peer at the scans, and even our untrained eyes can spot that her jaw is rather misshapen, and definitely not symmetrical.

12 noon or so, and we then have the wait for her to come round. About 2 o'clock and she stirs and demands a drink. By the time I come back with it, she's back out and doesn't stir again until nearly half past three.

I have to go to the car park to renew our parking ticket, and I come back to find an empty room. A nurse pops her head in to inform me that they are in the TV lounge meeting characters from the kids TV show "Polka Dot Shorts", who are appearing on stage somewhere locally, and having pictures taken by the local press! Even in her still dozy state she manages to get into several of these photos (one of which appears the following week in the local newspaper.)

Finally she stuffs down several slices of toast, a lot of drink and some chocolates, the needle is removed from her hand, and we are off home.

Hopefully the next instalment will reveal the results of the CT scan and we will be a bit more aware of precisely what needs to be done.

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17 March 2001: Heather was supposed to have had the operation on Wednesday (14th) but thanks to an administrative cock-up at the hospital, the anaesthetics department did not know that Heather was a special case until the afternoon before the operation. Unfortunately they had no-one available with the necessary qualifications, so having spent an entire day at East Grinstead, and having prepared Heather for what was to happen we had the rug pulled from under us. Heather was upset, as she was fully expecting to stay there and have the operation.

Things have been rescheduled for 2nd May, and I have personally written to all the departments involved to let them know when we are coming, so providing Heather does not have flu or a rotten cold the operation should go ahead then.

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30 April 2001: Operation postponed again (actually 2 weeks ago) This time I am not particularly annoyed as it was due to an urgent cancer case coming in. The new date will be 23rd May, and hopefully it will all happen then. The surgeon is now to be Mr. Sneddon instead of Mr. Lavery.

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23 May 2001: Here we are again saying that the operation has been postponed! Mr. Sneddon wants to do an alternative operation (distraction) that doesn't involve the grafting of a rib. The principle is similar to that used when lengthening legs - the bone is broken and held apart with a bracket and the bone then grows back together. The process takes about six weeks, as opposed to a year for the originally planned graft. The surgeon went to Great Ormond Street Hospital on Monday to get the device and talk to their consultants. It turns out that this operation has only been done six times in the world on a child of Heather's age, consequently the operation is now to be performed at Great Ormond Street. The operation would not have happened today anyway since she has had a mild streptococcal infection and so they would not have gone ahead.

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25 June 2001: We have an appointment for a consultation with new surgeon, Mr. Newman, on 4th July at Great Ormond Street. Thankfully, the rail strike planned for that day has been called off!

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4 July 2001: What a horribly hot day for travelling about on London Underground! Found GOSH okay from Russell Square tube station, went in more or less on time. Heather didn't much care for the examination room (she seems to have a thing about dentist chairs.) The upshot is that the operation is now going back to being a costochondral graft and it will take place on October 4th at Great Ormond Street.

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7th October 2001: Finally! It's been done! We went up to Great Ormond Street Hospital on Wednesday 3rd October, driven there by an ex-ambulance driver (thanks, Alan) and dumped our bags in Peter Pan Ward and then had to go straight down to the Maxillofacial Department to see the surgeon (the fourth one, the previous GOSH has moved on although he did come back to help out in the operation.) We went through all the required pre-operation procedures - blood tests, x-rays and photographs before finally being left alone to play in the ward's playroom.

Thursday morning, and things are due to start around ten o'clock - as it turns out that is when she is given the pre-med. About an hour later she is wheeled down to the theatre where we stay with her while she is anaesthetised, which has to be one of the most horrible experiences I have ever had to endure because she finds it extremely upsetting, despite it having been explained to her before. Once she is under, we have to leave while they put in anaesthetic tubes, so we are given a pager (so that they can contact us when she is in the recovery room) and we wander off, have dinner (even though we don't feel like it) and return to the ward and hang around until quarter to four when we are called down to the recovery room (she's been out 15 minutes), and we wait for her to wake up a little and for her after-operation x-ray to be checked - the pleura (lung lining) had been slightly torn and stitched up, and it had to be checked. It's all okay and we return to the ward. Usual after-operation stuff, that is vomiting, crying and so on. It's not too bad, in as much as we have been more-or-less expecting this. There's not much to do as most of the time she is asleep. Mr. Lloyd the surgeon comes round to check on the patient and he seems pleased - the operation went as expected. Christine goes to bed around 10pm and I push off to my hotel around 11pm (encouraged to do so by the night nurse!)

Friday: grumpy, tired and hungry. We spend the morning trying to get her to drink. Needless to say this annoys her, and eventually she tells me to go away (or to be more accurate she tells me to "bugger off" *ahem*), which I do as I know that someone is coming at 3pm to remove the drain tubes from her head. As this is the sort of thing that results in me feeling somewhat queasy and faint, it is best that I am not around - they don't need a wussy adult around hampering proceedings! When I get back, it is to find her sitting in a chair drinking and munching her way through two tubs of ice cream. Still grumpy, though, especially when the nurse comes to remove the tube from her chest. She does manage to walk to the toilet and back twice after she has had a little bit of dinner (chips and spaghetti) so her strength is returning. Off to sleep at normal time, and we go for a curry!

Saturday is a vast improvement. Reluctant to eat and drink first thing in the morning but soon she is encouraged to eat something. Tries a Farley's Rusk in milk which she does not particularly go for, so she has some toast on which she has a good go at chewing. This might not sound like much, but after years of not being able to open her mouth, chewing is a major step forward. In fact she eats and drinks so much during the morning, and looks so much better that we are discharged; free to go as soon as the tubes are removed from her hand and the foam block from her ear. One last meal at the hospital, a final nurse-versus-Heather battle, and we are on our way out of the door.

Although it was obviously uncomfortable at first, she is starting to open her mouth. The easiest way to get her to do this is to ask her to stick her tongue out, but now laughing and eating are encouraging her to do more.

A note on the possible cause: when she was two weeks old, Heather had an infection that led to an abscess around one of her ribs. It is apparently most likely that this also manifested itself in her jaw joint affecting its growth, but we still have no idea of the cause of that infection, so it will have to remain one of life's little mysteries.

There are, of course, going to be follow up visits to monitor her progress, the first of which is on this coming Wednesday (10th October).

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16th May 2002: Last Tuesday we went for a checkup at GOSH. While we were expecting to meet the new surgeon, Mr. Lloyd who did her operation was there. They are very pleased with her progress (to the point that we suspect she is healing far better than normally expected.) Back in November for a further checkup when they will take x-rays for her records, and thereafter annual checkups. She will probably require a brace to line up her teeth when she is about 9, and if they do need to do any further operations they probably won't be until she is about 16, so we can relax a while!

Met up afterwards with a friend who works at the Royal Opera House, and took us on a tour round the new opera house. Having seen the Royal Ballet rehearsing, Heather is now obsessed with ballet!

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11th February 2006: So this year's annual check-up went okay. Just photographs taken and talk of wanting to do more surgery to straighten up the growth before the next major growth period, using the distraction process mentioned a couple of years ago. We may be a little stymied on that front as it looks like Heather may have a kidney problem that needs to be resolved first. Tests for that in a couple of weeks. In terms of the jaw, though, it is functioning well and she has a decent opening - it works just fine, judging by the amount of food passing through it lately!

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13th September 2006: Just a quick note to say that the kidney stuff is comparatively minor and not going to affect the jaw stuff! Next checkup at GOSH is in December to check on Heather's growth to see if she will be ready for the next operation in summer 2007 as it is best done after the growth spurt that occurs around the age of 8 or 9.

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2nd January 2007: December's check-up was brief. It appears that distraction will almost certainly happen in 2007, so we have been booked for a CT scan in February so they can see precisely what is going to be required. We are hoping that she will have the internal braces, so that there is minimal external ironmongery, but the bone has to be a particular shape for that (fortunately, the opinion at the moment is that she will be able to have the internal one.) We will be seen in the Maxillofacial department in March for further details.

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5th September 2007: The last three weeks have been hectic. Heather had the operation to install the distractors on Thursday 16th August. The operation went well, although it was apparantly quite tricky. We took her down to the theatre at about 2:15 and were called to the recovery room about four hours later. Not unexpectedly she was very swollen around the lower face, and was sick a few times through the night. She was attached to a machine that would administor controlled doses of painkiller if she pressed a button, which she did not use as much as some children apparantly do. She has been fitted with the internal distractors, with the handles poking out behind her ears. The handles have an hexagonal head which is turned by a hexagonal screwdriver. Each turned stretches the distractor and therefor the lower jaw by half a millimetre.

Friday was a slow day as she was still obviously distressed and still unable to take in fluid other than the occasional squirt of water from a syringe. She would not talk in more than a murmur as it was obviously uncomfortable to move her mouth. By the end of the day, the machine with the painkillers had been disconnected and she was taking paracetomol and ibuprofen orally. As she was still not drinking, she was placed on a drip to keep her hydrated. She was also dribbling a great deal as her mouth was still numb and she could not really feel the saliva.

Saturday was a bit of an improvement as she managed to get out of bed, but she still was not drinking, but it seemed more unwilling than unable. She had been told that if she wanted to go home, she had to eat and drink something but she still largely refused, and although she was a bit brighter by the end of the day - she managed to play BopIt for a while (with one hand as she refused to use the hand that had the canulars in) - she had not drunk enough to be allowed to go, which upset her a bit but brought it home to her that she had to start eating and drinking.

Sunday morning she managed a bowl of milk-soaked Weetabix, and two or three glasses of drink and we were discharged by lunchtime. Still swollen and mumbling but now eating and drinking. The dribbling lasted another day or two.

I must emphasise at this point that what is described in the following three paragraphs is not normal - it is the first time that GOSH had ever seen it happen. If you or your child is about to have this done please bear in mind that the chances of this happenning again are so remote as to not be worth worrying about.

Tuesday was the first follow up appointment when the screws were to be turned for the first time. The screw on the right jaw was turned and did not cause much by way of pain or discomfort, but the left did seem to smart a lot but she recovered from that quite soon.

Wednesday was the worst day - we turned the screws in the morning, again the right hand side was comparatively fine but the left obviously caused a great deal of discomfort, and in the evening she was quite hysterical at the thought of having the screws turned, to the point that it took two hours to get her to sit still. Turned the right, no problem, but the instant we turned the left she screamed at which point we decided that we needed to talk to the hospital, and as it was by now 8pm we decided to defer it until the morning.

Friday. Needless to say that after this experience she was less than happy about the idea of turning the screws again. We insisted that someone from the department was present when the screw turning was started again. Until that point we don't think they had fully understood just how extreme her reaction was likely to be. They spent twenty minutes trying to calm her and reassure her but it was no good, so it was decided to defer it until later in the day so they could devise a strategy. At this point we will make a special mention for Jenny, the psychologist on Peter Pan Ward without whose help we would have found the day far more difficult than it already was. To cut a long story short, after a mild sedation to calm her down the right hand screw was turned without distress. It was decided that the left screw would not be turned until the follow-up appointment on Tuesday.

On Saturday, Sunday and Monday we turned the right screw once a day. On Saturday it took about half an hour for her to let us turn the screw and by Monday it was down to about ten minutes as she gradually realised that it didn't hurt much at all, other than making her teeth 'jangle'. Also by Monday she was pretty much her usual self, other than being somewhat tired!

Tuesday. It was decided that she would me mildly sedated again when it came to turn the left screw as she was understandably anxious about it being turned again. While on Friday it had taken half an hour to cajole her into putting the gas mask on, this time she was straight into the dental chair and had the mask on before either of us were in the room! About five or ten minutes later she was relaxed enough to let the surgeon turn the screws.

Over the last week things have progressed to the point that she is quite happy to have the screws turned, and even her teeth don't particularly jangle now either. The process of turning both screws now takes little more than a couple of minutes.

Physically she is now showing signs of the jaw having moved forwards - her eye teeth now fall behind her bottom lip when her mouth is closed, and the lip is now resting against the back of her front teeth instead of about half a centimetre behind them. By next week we expect that they will pretty much inside her mouth where they should be. Her mouth capacity has now increased enough for her to be able to get a dessert spoon into it, something she could not quite manage before. And she is happy as that as the days go on she looks less and less "like a hamster"!

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8th February 2008:

The distractors were removed temporarily in November - it was hoped that the procedure could have been completed by Christmas but it was not possible to re-site the distractors to allow for further stretching so they were taken out. To help the bone consolidate in the right place, metal plates were attached to her jaw to hold it in place. Now that the swelling has subsided, the improvement is very apparent. There is still a bit of an overbite but it is now far less pronounced that before. Movement is normal and mouth capacity is much improved (shorter meal times!)

The next stage will commence towards the end of February.

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