Welcome to Alyssa's blog ...

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Central Scotland, United Kingdom
My name is Moira, and I hope to share with you what my daughter's life has been like so far ... so you can all truly understand and appreciate the gift of pain, which we take very much for granted! Alyssa does not feel "peripheral" pain, which means she does not feel pain anywhere other than internally. This has led to many unintentional injuries and self-mutilation. My aim is to not only find others like Alyssa, and help those who may be going through what we are, as well as raising awareness about this condition, and how feeling pain is actually a GOOD thing! I am thankfully now part of a support group run on FB which is an amazing group of people, who all have varying types of experience with pain insensitivity. I can be contacted directly via understandingalyssa@hotmail.co.uk

Self-injuries to date:

The following will give you some idea of what Alyssa has already done to herself ... so far!

* Knocked a few of her own teeth out while "teething"

* Caused huge ulcerated sores in her mouth, from "rubbing" her teeth on her tongue and inner cheeks

* Bitten straight through her lower lip - didn't even flinch!

* Chewed the end of her tongue off, resulting in emergency repair and incisor removal

* Had tongue repaired, then began chewing the side of her tongue as soon as her molars erupted

* Chewed a finger almost down to the bone

* Torn entire patches of skin off, and is scarred fairly extensively as a result! :-(

* Broken both feet - and I had to argue with doctors for almost 10 weeks with one of them, because they didn't believe it was broken! Even a lot of doctors haven't heard of Pain Insensitivity!

* Broken her left leg, just under the knee, and walked about on it quite happily. Took us at least a couple of days to realise, because we aren't entirely sure how she broke it. And it didn't give way until after she'd already been telling me - in quite a bemused voice - that "her leg was moving, all by itself!" I now know that is Alyssa-speak for 'something is broken! Get X-rays immediately!'

* As a result of the biting injuries (and much deliberating!) the decision was made to remove all of Alyssa's baby teeth, as and when they grew in. This occurred up until her very last molars, when it was decided that we should try "crowning" her molars. Thankfully, it did the trick. And she got to keep her baby molars. We managed to save her upper adult incisor, but she now has a new lower incisor, which is causing a lot of problems. She has had all the dental assistance that she can really have, at this point, so now we wait.

* Required spinal surgery to correct a vertebral slippage issue, which she was completely unaware of. The op itself was pretty straightforward. The post-op period was lengthy, and anything but fun

* Developed septicaemia from one of her many episodes of cellulitis (usually from tiny wounds she doesn't know she has, which bloom into cellulitis quickly), because nobody realised it hadn't gone away, and was just grumbling away as an abscess in her elbow. When she collapsed, it was scary!

* Managed to dislocate her left hip, falling from her trike .... but it took us 4 months to realise, because she didn't feel it!

Wednesday, 15 February 2017

Update 3 of 3 - the 'fully medical' one ....

When we returned from Florida, we saw Alyssa's 'general' orthopaedic surgeon (ie not her spinal surgeon).  We had an in-depth discussion about whether to actually fix her Left hip or not.  Her ortho explained that it would be a very big operation and we'd probably be looking at approximately a year of recovery time.  Needless to say, he was not keen to proceed with it unless it was deemed to be vital to her health.  I was concerned about the implications of not having her hip relocated, especially as far as how it might affect her present spinal issues.  She already curves to that side with her scoliosis (which we are already trying to correct/reduce with her spinal brace) and her L leg is longer than her R leg, due to it being out of joint.  She therefore walks with a very exaggerated pendulum gait; ie swinging from side to side as she lifts each leg.  She can control the swinging to some extent if she is reminded to "not walk like a penguin" but some of it is unavoidable.  I told him that although I did not want her to have such a big operation, we had already discussed with her spinal surgeon that the spinal brace was required in order to avoid major corrective spinal surgery.  The spinal operation would be much more invasive and serious than any hip surgery, with expected complications and repeat surgeries, before we even got to the healing and recover process.  It is a possibility that - even with the scoliosis brace - she will eventually need to have this operation anyway, but her spinal surgeon and I are keen to avoid it for as long as possible.  Having her hip out of joint not only weakens her pelvis (and therefore affects her ability to walk, because her core strength is reduced), it affects her posture, which in turn aggravates the scoliosis.  He decided that he would prefer to speak to another specialist and get his opinion on Alyssa's overall issues, and how doing the op / not doing the op would affect her in the longer term, and said that the new specialist would probably see Alyssa for himself, so he could assess her properly.

We didn't get as far as meeting the new orthopaedic specialist before she developed pneumonia in mid-December and had to be hospitalised for IV antibiotics and fever-reducers.  She had gotten increasingly breathless as the weeks passed after our return from Florida but none of the docs had heard anything to concern them when they listened to her chest, so it wasn't obvious until she was pretty poorly.  I'd even had her tested for anaemia, in case that was what was affecting her breathing, and causing her to fatigue so quickly.  On the days where she actually made it to school, they were having to send her home by about 11am because she was just so tired and pale.  She fell asleep in class on more than one occasion, which tends to happen frequently if she's ill.  They usually let her nap for 15-30 minutes to see if that is enough to 'recharge' her, but any more than that and they know she's unwell, and needs to be in her bed.  She was having to sleep for at least a couple of hours at home, before being able to get up and engage in any form of activity, then going back to sleep at bedtime and sleeping all night.  So the pneumonia was good, from the aspect that at least we knew what was wrong with her, and could begin treating her.  She recovered fairly well from that and was discharged a few days before Christmas.  

I was hoping this meant that we'd dodged an illness over Christmas bullet, but I then had to take her to A+E on Christmas Day because she scratched a huge area of her eye surface (corneal abrasion)!  Something must have irritated her eye and she'd then made it much worse by continuously rubbing at it because it was "itchy" until she couldn't see out of it any longer (and even then, she was still attacking it!).  The A+E doctor was fab, though said he wasn't sure how to proceed, because he'd never dealt with a corneal abrasion so severe in someone so young before.  The abrasion was covering the entire pupillary area of her cornea.  He asked the on-call opthalmologist to see Alyssa and she was prescribed some antibiotic ointment.  We were asked to return to her normal priority eye clinic a couple of days later for a re-examination, which we did.  I was very worried about her sight because we've always been very lucky with regards to her vision (numerous children go blind, or partially blind, from not feeling pain, because they stick their fingers in their eyes as babies, or get particles of dirt etc in their eyes and don't feel it.  They then continue to rub at them, until the eye surface is so damaged that the damage can be irriversible.  Alyssa had managed to have perfect vision and fairly healthy eyes, with only a couple of minor abrasions in the past few years, and I was hoping this would continue.  She's now been for multiple eye checks with various eye doctors and then with her own, and the abrasion has mostly healed, thought there is some scarring to the surface of her eye.  The opthalmologist is unsure if it will resolve completely, but she is hopeful.  I mentioned that my new concern that Alyssa had developed a "squint" (lazy eye) since the eye injury in December.  She tested Alyssa's eyes again and said she agreed, and that we should attempt to correct it as soon as possible.  It is most likely due to her having been unable to see for a period of time, and losing focus in it, because she was using the other eye to compensate.  The optometrist was unavailable that day so we are back on Friday morning to have a full eye test done, and to find out if Alyssa will get glasses with / without a patch, to make her concentrate her focus in that eye, and strengthen it again.  

Alyssa's itching had also increased severely during November and into December, so her dermatologist (who is brilliant!) prescribed a new drug for her to trial.  She was constantly coming home from school with her shirts more soaked in blood than normal, and she literally tore her neck to shreds at one point.  She is somewhat of a guinea pig when it comes to medicines because she is an almost unique little entity so her doctors are learning about the condition from her!  Medicines do not always work the way they should, because her nervous system does not work properly, so a lot of the time it is 'educated guesswork' as to what might help ease or at least reduce her symptoms.  She has been on many drug trials in her life and it seems likely that it will be the case for some time.  The new drug had to be put on hold when I realised she had pneumonia however, as I didn't want her starting a new drug when her immune system was already under stress.  Once it seemed that she had the pneumonia under control, she developed cellulitis again and had to be admitted to hospital for further IV antibiotics.  We only managed to get approximately 48 hrs worth into her - over a period of about 3 1/2 days - because her veins were so difficult to find.  Her insensitivity to pain means that she is not careful with IV cannulas even when they're placed in her arms or hands easily.  You or I feel a certain amount of discomfort if we lean on a cannula, or use that arm/hand once one is in our vein but she feels nothing from that.  So she will lie on them, twist her arms into random positions, and sleep on them.  It doesn't take long for them to bend inside her veins, and become useless.  She was discharged before the infection had resolved due to this issue as there was no point in keeping her in hospital if she was taking antibiotics by mouth, and we had really run out of options for IV's when she wasn't sick enough to warrant much more invasive alternatives.  So although her arm was still hot when I left (and I was uncomfortable with leaving with it still hot), we really had no choice.  She continued the antibiotics for a week, then her dermatologist prescribed a longer term course of prophlyactic (preventative) ones.  She was then unable to start those when she should have either because I realised that the pneumonia had not quite gone away and had flared up again, so I had to abandon the prophylactic antibiotics in favour of ones more targeted to chest infections.  It seems to have resolved finally, so she has now started the longer term ones, which will hopefully prevent her getting cellulitis again.  She has also started the newer drug trial for the excessive itching, though it is too early to say if it is working or not.

The hip:

I saw the new orthopaedic specialist last week about Alyssa's dislocated hip.  He had both hips x-rayed again in very specific views and compared them to her October ones, then physically examined her.  He was visibly stunned that he could put her L hip in and out of joint without her even caring (she did cry that he was holding her knee too tightly, and she was quite upset about that!)  As soon as he loosened his grip on her knee, she didn't care at all.  That blew his mind somewhat.  It's one thing to know about Congenital Insensitivity to Pain .... it's another thing completely to witness it for yourself.  He'd obviously been told about her and had read her notes, but it hadn't completely prepared him for the real thing.  He just kept saying "That's crazy!!  That should be extremely painful!"  I accompanied him to the consultation room to discuss his findings.  I explained that her regular ortho had been very reluctant to go ahead and fix her hip for the reasons mentioned above, but that I was concerned about the problems of not doing it.  He said that sadly her spine didn't even really come into it because the damage already done to her hip is very clear.  The x-rays she had done in October and the ones she had last week show significant erosion and soft tissue damage from her leg bone grinding against her pelvis.  Her abnormal gait (worsened by the dislocation) as well as her abnormal sitting/lying positions mean that her hips are forced into positions they would not naturally be in.  There is really no doubt that her hip needs to be surgically fixed back into her joint in order to prevent further damage.  Unfortunately, the x-rays also show that her R hip joint is not great either, and the ortho doesn't feel that it would take too much for her to dislocate that one as well.  :-(  He asked whether I thought we should fix the dislocated hip first; let her recover, then go back in and stabilise the other one, or just operate on both hips at the same time.  I immediately said that we do both at the same time.  It will be a huge procedure, but the dislocated hip procedure is already huge.  By doing both at once, it's all over in one big operation, there's only one recovery period that she'll have to go through, and she'll only have to deal with one cast post-operatively.  The ortho said that he also felt it was best to just do both at the same time, for the same reasons.  He said that he would also request a CT scan of her hips, so he can determine how much soft tissue damage there is to her L one, and also assess her R one at the same time.   

I do not have a set date for her operation but it is to be at the end of April.  The procedure will involve cutting a small section of bone out of her L thigh bone to aid the curving of her leg towards her pelvis.  The socket of her L hip is not very curved so this will be reshaped, then she will have her hip put back into place and held together with metal plates and screws.  Her R hip will also be stabilised surgically to prevent it from dislocating at a later date.  She will then be put into a Hip Spica cast made of plaster for 6-8 weeks, to keep her hips in the correct position while they heal.  She will be unable to stand, walk or move about by herself, so that's obviously going to be really hard for her - physically and emotionally.  The cast itself will be a nightmare for her (and me) because she is so prone to pressure sores (which she won't feel) and infections, not to mention that it will fatigue her upper body, just sitting in it.  It takes 4-5 HOURS to apply the cast, so it will be put on while she is still under the General Anaesthetic, but normally in a child with CIP, it would be changed frequently to check for wounds from rubbing.  She had pressure sores very quickly from her leg cast, and she wasn't able to walk on it at all - she just wiggled her foot inside it, because it didn't hurt.  This cast will cover her entre pelvic area, and she is bound to wriggle about inside it.  We won't be able to check the state of her skin without removing the cast, which would require a GA each time she needs one put on.  I believe that a GA is not normally given when removing the hip spicas but she reacts very badly to the vibration of the saw, and then immediately starts trying to destroy the skin which has been covered by it.  The air getting at her skin after it being inside a cast for so long is absolutely unbearable for her, and she will tear herself to shreds if we cannot stop her.  I will discuss with her ortho what the best plan for cast checking and removing, as we get nearer to the operation.

We do not expect her to be able to walk for a very long time after her cast is removed but hope that she will eventually get there.  It can take a child of normal strength and ability up to a year to walk properly again, but I expect Alyssa to take longer than that.  It took her until she was almost 2 years old to take her first steps, and I believe it is going to take a lot of work to get her walking again.  Her physio is concerned about her rebuilding her strength afterwards because she didn't lose any muscle when she was fitted with the scoliosis brace, and she couldn't walk as soon as it was put on her - because it changed the angle of her pelvis.  This will obviously be much more severe a process.  I am having to consider things like a special bed, hoists, a special car seat, whether her current wheelchair can be adapted to accommodate the hip spica, and a whole other host of things that are a bit overwhelming at the moment.  And I have no doubt that there are many aspects I haven't even thought of yet, nor that she will prove to have her own factors to add to the mix.  The year ahead is going to be challenging, to say the least.  

I have been asked what she thinks about the operation.  In all honesty, she doesn't really comprehend what's about to happen to her.  She knows she's having an operation.  She knows she's going to be put in a cast for 6-8 weeks.  And she knows that she's not going to be able to walk, or stand, and that it will take a long time for her to manage those things again.  What she understands is a completely different thing.  As adults, you or I might know that we're going to be put in a cast, and comprehend the implications of it.  But we still wouldn't really come to grips with all of the things which will be affected by it until it happens.  She is 9 (10 next week!) and doesn't have the capacity to foresee half of what I do, and she'll wake up in a 'sitting position' in a solid cast.  That in itself is bound to be overwhelming, before coming to grips with an entirely new lifestyle.  I'm not even sure yet how to help her cope with that, but we'll just have to get through each day the best way we can!  And I know we have a load of people who will spur us on, and help us get through those days.  She is having the CT scan on Friday so I will hopefully know more in the coming weeks, and be able to plan out the basics.

And finally: she is ten next week!  TEN!!!  Her life has been very unfair (and cruel!) a lot of the time, but she is a pretty amazing little being.  Despite everything she has to go through, she has the most brilliant personality and sense of humour, and still manages to live in her little 'pink and princess' Disney bubble.  She is very excited about going shopping for her birthday next weekend "on the train!!!!" and we will no doubt come home with yet more toys that she doesn't need, as well as clothes that she does!  But there are definitely worse things that she could be excited about.  Whatever she finds that I think will make the coming months easier, we'll be buying!  

As always, thanks for reading.  I always pass on messages and comments to her when they're left (on here, or on Facebook) after people have read it.  They make her smile :-) 

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