A quick update before hospital this afternoon ...

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" and 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. After having the tip of her tongue repaired, she 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 for at least a couple of days. We'll never know how she broke it. Any time she says "my --- is moving, all by itself," we get x-rays done!

* She had to have all of her baby teeth removed, as and when they came in, due to all the biting injuries.

She is still dealing with the after-effects of that, as a teen.

* 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 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!

* Had corrective surgery performed on both hips. Unfortunate complications ensued, which eventually caused the entire removal of her Right hip, and part of her femur.

Thursday, 1 October 2009

A quick update before hospital this afternoon ...

Only have a few minutes but thought I'd give a quick update while I can - so I don't forget to put something else that the joy of Pain Insensitivity brings us!

We've spent the last however many months trying desperately to get Alyssa to drink juice ... because she gets medication that is more easily given if disguised in juice (she can taste it in water and won't drink it then!) so we've been letting her drink "Fruit Shoots," which are essentially little pre-filled bottles with sports-drink type caps on them. Now normal children - ie with teeth actually use their teeth to open them, but Alyssa doesn't have any at the front of her mouth at all. She only has the four last molars left, so she uses her gums to open them. A long time ago when we were seeing the SaLT (speech and language therapist), we were told that many children had all of their teeth removed, and that they learned to eat/drink etc perfectly fine because their gums just hardened up in place of the teeth. And - from my own experience as a veterinary nurse (which now seems like another lifetime ago) I know that we've sometimes removed all of a cats' teeth ... and they go on to eat dry food just as well as they did when they had teeth.

However; either Alyssa has had a "weakened area" at the top of her mouth where her teeth were extracted, or it just hadn't hardened enough .... and I discovered to my horror on monday that she has been gouging a hole in the roof of her mouth, opening the bottles! I am not a squeamish person (a good job, I hear you say!) but I was actually gagging when I saw the crater she'd created. She was - as usual - completely unconcerned and unaware that there was even a problem. I only noticed because I happened to glance over at her, and saw the blood running down the top of the bottle as she drank!

Needless to say .... Fruit Shoots (and any equivalent) have now BEEN BANNED!!! :-( Thankfully, Alyssa is still accepting the juice from within them from her cup (she wouldn't do that before) so she chooses which colour/flavour she wants in the morning now, and then watches me pour it into her cup and stir it (while I mix it with the powder that she's not really aware of!) ... then takes it back through to the livingroom and drinks it all. Phew!

I have the hospital this afternoon, where I'm seeing the Specialist Anaesthetist / Pain Specialist and I'm really hoping he now has a surgery date for me! I was promised that it would be done in "plenty of time before New York" and I now have three weeks to go. That may still be three weeks to everyone else, but I have a zillion things to do before being ready and organised to go! Travelling on an 8 hour flight would be challenging enough with any two year old, but with Alyssa it's going to be verging on the almost impossible - and I need as much as time to plan for that as possible! The surgery will either help her or not, and I need a bit of time to guage if it's had any effect or not ... because that will also affect my flight plans. Not to mention I'm still trying to sort out accommodation, find out what documentation I'm going to need for her to actually travel and find out exactly what the rules regarding medication are!

Then there's the packing - in between the fundraising events, medical appointments and her usual daily stresses to deal with - as well as remembering all the "little things" I'm going to have to make sure we have with us, because of her unusual severe aversions to things. Aaaaaaaaaaaaaarrrrrrrrgggggghhhhhh!!!

Ok, baby steps! Hospital this afternoon, find out details available now ... and take it from there! Thanks for reading!

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Background...

Alyssa's story is a very long and complex one, and to put it all would require actually writing a novel, so I started this blog as a way to try and document some of what it is like to live life in this way. And so anyone reading can get an insight into what it is like to live a life without normal pain sensation. I will attempt to update it regularly, though our lives can get really crazy, as most of you followers already know:

Alyssa suffers from Hereditary Sensory Autonomic Neuropathy (HSAN) Type 7 - also called Congenital Insensitivity to Pain (CIP) with Dysautonomia - and is one of the rarest conditions in the world. It is actually a very complex condition, but in an attempt to put it simply, she does not feel pain in her arms, legs, head, fingers etc. If she hurts herself; she is not aware of it, and is usually fascinated by the sign of her own blood! Which is not great! As well as constantly having accidental injuries, she will self-mutilate - because it doesn't hurt, and well ... because she can!

How it all started: She was not the "typically happy" baby that is usually found in a child who does not feel pain, and we now know that is because she suffers from frequent bouts of abdominal pain. She can - and does - feel visceral (gut) pain, and we have trialed various medications in an attempt to reduce this. Sadly, the pain which she does feel is not 'normal' pain either, so very difficult to treat. Her nervous system just does not work like most people's. There have been 2 theories regarding the gut pain - that it is more intense because she has nothing to compare it to, not feeling pain like everyone else does; and the other is that her visceral nerves are working in a directly opposite sensitivity to her peripheral nerves. Neither has been made clear, although she has definitely been feeling obvious discomfort when she something swollen - which at least makes it easier for me - because I know something is wrong much quicker these days. I don't always know what is wrong, but can hopefully narrow down the "body part" search.

Alyssa has another unusual factor for a child with one of the pain syndromes, because she has an intense "itching" sensation, which causes her to tear and friction-burn entire patches of her skin off. As she doesn't feel any pain from this - even once the flesh is entirely exposed - she doesn't learn that she shouldn't do it. As a result of this, she is very badly scarred already. She had an experimental drug trial of Thalidomide, which didn't seem to make much of a difference ... until we stopped it! It became very obvious that it had actually made a significant improvement to her overall health, in a roundabout way, and reduced the itching, by reducing the excessive sweating that is also part of her condition. Within a few days of coming off it, she was miserable, drenched in sweat, and tearing herself apart again. She had multiple wounds which became infected very quickly due to the excess sweating in them, and she tore off any areas of skin she could easily access. A few days after being back on it, she was much more settled again, and she has been on it ever since. We are about to trial her off of it again, after her being on a different drug for a few months now, and seeming fairly stable. Time will tell how that goes...

Alyssa does not react to things which you or I would find painful - such as being cut, bitten, or fracturing a bone ... but she reacts to heat and cold sensations as if they were excruciatingly painful! When she fractured her L leg at 4 years old, we didn't even know for a couple of days, because she continued to walk about on it, happily. The orthopaedic doctor was stunned to see her wander about on a leg which he discoved was broken, but scream when he tried to touch her ... because his hands were cold! His face was a picture. One we have seen many times since then! My girl is pretty unique!

Anything which you or I would find simply 'cool' or 'warm' will be either freezing or burning to Alyssa. And you do not realise how many things have a 'temperature' until you have a child who reacts to almost everything touching her skin. Almost everything which isn't fabric is an issue for her, which causes a great deal of distress and difficulty in day-to-day life.

For those researching pain insensitivity ...

The pain disorders can be very confusing, and even those of the recognised syndromes have many different names.
For those people (and to help my page be found via search engines so I may in time find others for future contact) I shall list the many searched terms:

HSAN - Hereditary Sensory Autonomic Neuropathy
CIPA - Congenital Insensitivity to Pain with Anhidrosis
Congenital Indifference to Pain
Congenital Analgia
Congenital Analgesia

There are - of course - many others, but those are the most commonly searched terms. The rest are usually only used by the medical community and they generally use HSAN too.

Understanding Alyssa ... and Pain Insensitivity - Life Through Her Eyes

Welcome to Alyssa's blog ...

Self-injuries to date:

Thursday, 1 October 2009

A quick update before hospital this afternoon ...

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For those researching pain insensitivity ...

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