How things are today ...

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.

Tuesday, 1 September 2009

How things are today ...

It's been a pretty bad weekend as far as gut pain goes, and poor Alyssa's been complaining fairly constantly about her "sooo" (sore) tummy/bum. It's still causing her pain for up to 2-3 hours before a bowel movement and sometimes afterwards as well, if she hasn't passed enough. So ... pretty much most of her waking (and some of her non-waking) hours! *sigh* So much for Pain Insensitivity!

Saw the dentists today and we discussed the large ulcerated area on the inside of Alyssa's cheek. The other side which had a small area starting appears to have almost healed again, and the large one is looking slightly better than it was on Thursday. So we had yet another debate about her teeth. The dentist feels it has to be a joint decision (which I'm glad about, but it would obviously be easier if it wasn't MY decision in any way!) so we're going to leave things for now and have her seen again in another few weeks ... unless she decides not to wait that long! I just can't bring myself to remove them at the moment when - gut pain aside - she's eating so much better because she can actually chew most things now with them, and I really don't want her going backwards. Each time she has teeth, she learns to use them (which sounds really obvious, but it is a major thing for her ... she keeps learning to use teeth then has them removed and learns to use her gums alone ... then we start all over again!) ... and we go through the gagging/choking cycle again, because she's used to just shoving stuff in her mouth and leaving it up to the teeth to sort out - like she should be able to.

I am not as bothered by her chewing the insides of her cheeks as I am about her chewing her tongue ... that's not to say I'm NOT bothered by it! I just have to balance everything up - constantly! If this can be passed over as yet another "accident" .. or she's chewing them again because she's biting down due to the gut pain ... then I have to try to save them, because overall she's better with them. If she's going to continue to destroy the inside of her mouth with them, then the ulcerated areas will become infected and that will change. But the decision is harder to just say "get them out" when it's gradual like this. If I discover her - AT ANY POINT - chewing her tongue, then they shall be removed as soon as is absolutely possible! As awful as it sounds, it's easier to make such a drastic decision when the potential damage is just as drastic!

None of this is fair! :-(

I also caught up with the neurologist, but sadly the anaesthetist was held up in theatre (although it's his busiest day and I knew there was the possibility that would happen!) and we had a bit of a chat about what's going on with her gut pain. Thankfully (for once!) Alyssa actually demonstrated fairly well the level of pain she's in ... instead of just "dealing" with it like she usually does on hospital outings! Why do they do that?!?!

We discussed a procedure which would hopefully help Alyssa's pain on passing bowel movements and I shall discuss it in more detail once the anaesthetist manages to call me (he's off on a wednesday, so I'm guessing it will be Thursday now before we chat). It will require another GA but there's the possibility of that with her teeth anyway, and it's hardly anything really that new to us. She's had more of them in her 2 1/2 years than most people have had in a lifetime! It doesn't sound particularly pleasant ... but anything is better than 2 1/2 years of chronic excruciating pain. It would be so nice to think she could be knocked out in pain, and wake up feeling none for the first time (after recovery time, obviously) .... but I'm also thinking that it seems too good to be true after all this time. However, if it even made life a bit more bearable for us then it's definitely worth it. And - if the teeth need to come out - at least they could both be done at the same time, so one less GA.

Will know more after speaking to the anaesthetist .... and will update. Thank you to all of you who continue to read! x x x

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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:

Tuesday, 1 September 2009

How things are today ...

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For those researching pain insensitivity ...

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