Life is a blur ....

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, 23 March 2010

Life is a blur ....

I've been packing all the non-essential stuff (how come you can have non-essential stuff ... yet still "need" it?!?) and de-cluttering my little head off!!! :-D

I've been in and out of the new house (with thanks to the very kind decorators, who simply took my word for it that it was about to be my new house) so have been seeing it as it develops. It's very cool. Alyssa already loves it, and it's not finished yet. She's been "hiding" in the cupboard of the bedroom that will be hers, and she just loves that she can wander from one room to the next - NO STAIRS!!!

We're going to a special hospice tomorrow, to let Alyssa try out a Safe Haven bed and see if she's a: tolerate the material against her skin (or if we'll have to have special covers made in order for her to be in it, and b: tolerate being in it and not being able to see out (it's pretty much a big pale blue padded cot, with a detachable side so I don't have to lift her in and out), but they're all custom made for each child and their particular issues so I'm still hoping that some kind of perspex/plastic window could be put in. It's essentially a mini room, that she would be able to sleep in (freestanding, or raised - our choice) without falling out, hurting herself on cot bars or standing up and falling over edges, like a normal bed guard. Will only know what she thinks of it once she tries it tomorrow, but she's pretty excited about getting to try out a new bed! ;-)

The OT (did I mention I love her already!) has offered to come out to the new house once we're settled, and help me design the special sensory room - with equipment that will specifically help Alyssa, and her needs!!! Alyssa is VERY happy with this, because she's in love with the OT lol! Can't wait to have her over to her "nooo houuuuuuuuuuse" ;-)

Alyssa's guts seem to have settled down again now that she's had the course of probiotics, so very pleased about that. Still doing the food diary though, to just see what types of things she is more sensitive to but glad she's back to "her" normal as I can adjust her medications accordingly if I know how her guts are going to behave.

Haven't really talked much about her actual Pain Insensitivity for a while, and her perception seems to be changing in some respects. She is now reacting strongly to anything contacting her legs and feet (things scraping/running across/being lightly bumped against) yet not reacting in the slightest if she falls and bangs her knees/ankles or feet. Most confusing! She screamed the place down the other day when I accidentally stepped on her foot (with no shoes on) and it took me almost 20 minutes to calm her down, but she fell the following day and collapsed on one ankle but didn't show any concern whatsoever - and I was worried she'd broken it! She still doesn't care if something cuts/tears/scratches her arms, hands, upper torso or head (unless she sees it happening, then she'll tell you - but not in a distressed manner, just as a "matter of fact") but the zip on her special suit hadn't been fastened properly yesterday when I collected her from the family centre. I didn't realise this and when I unzipped, it caught the skin on her thigh and she screamed before I even realised it had happened! Normally, I know it's happened before she does (sometimes I am the ONLY one who realises it has happened!), so something is definitely changing.

However; because she still doesn't react to things she SHOULD do - like banging her head really hard, falling and smacking her knees on the concrete, or landing on her neck and not complaining, I am convinced that it is a "skin sensation" issue - not a deep pain perception issue. I have e-mailed Dr Axelrod (the specialist we saw in New York) for her opinion on my confusing and conflicting child!!

Ok, that was probably short and sweet .... but have loads to do. Hoping to move ASAP!! :-)

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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, 23 March 2010

Life is a blur ....

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For those researching pain insensitivity ...

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