Pain Insensitivity

Thought that today; instead of just writing about Alyssa, I'd write about pain insensitivity and how it affects sufferers - pretty much regardless of what "diagnosis" they have, causing the pain insensitivity.

Keep in mind that although most seem to, not all suffers have total pain insensitivity - ie some only have lack of pain sensation towards the end of their arms and legs so they can feel pain in other areas of their bodies.

Likewise, sufferers like Alyssa can feel internal pain ... although they feel no external pain at all. Whilst - certainly in Alyssa's case - this can be seen as a curse as well, because her gut pain is almost constant (although now thankfully fairly well-controlled on Gabapentin) which just seems cruel; it is also hopefully a blessing! It gives hope that internal ailments which can kill sufferers of conditions like CIPA (Congenital Insensitivity to Pain with Anhidrosis) very easily - like appendicitis - will be detected earlier in these patients, and possibly prevent the fatality which discovering these conditions too late.

CIPA (also known as HSAN IV - Hereditary Sensory Autonomic Neuropathy - Type IV) sufferers feel no pain whatsoever, they do not feel the intense pain which comes from appendicitis and sadly; by the time the appendix has ruptured and caused peritonitis, which would cause more obvious signs of illness, it's usually too late to save them. I cannot even begin to imagine the mental torture that comes with having saved your child repeatedly from countless dangers, and from the self-mutilation ... then discovering there was an "invisible" time bomb ticking away in front of your very eyes!

The most common symptoms of pain insensitivity are:

- Multiple painless fractures; with or without poor healing. As fractures are not felt, they can go unnoticed for considerable time and lead to severe bone infections. Fractures should be suspected wherever bruising and swelling are present.

- Recurrent skin infections; both from injuries sustained from accidents, and also from self-mutilation

- Joint dislocations and deformities (Charcot Joints). Feeling no pain leads to overuse of bones and joints, as the sufferer cannot tell they are either using the joints incorrectly, landing incorrectly from sports or exercise, and thus they are not rested or corrected appropriately.

- Corneal ulceration. As sufferers who have pain insensitivity have anaesthetic corneas, they also do not feel any pain when it comes to their eyes. Rubbing - particularly in small children - is a major threat to the eyesight, and can cause ulceration and infection leading to blindness if not treated immediately. Sadly, many sufferers have reduced vision from pain insensitivity. Other threats to the vision are foreign objects (dust particles, hair, eye lashes etc) because the sufferer feels no irritation ... so does not realise that the object is there. This can lead to irreversible scarring.

- Self-induced oral injuries. Pain Insensitivity is especially difficult in infancy, especially once the deciduous teeth erupt, because the sufferers begin chewing on their own mouths. This can be either minimal or extreme. Some have bitten the ends of their tongues off or mutilated them beyond recognition, others have merely chewed "pieces" off before being interrupted. Ulceration of tongue, inner mucosa and lips are very common and many children have their teeth extracted in an attempt to prevent further damage. Some children knock their own teeth out, usually accidentally. Others will actually pull their own teeth out! The reason behind this is unknown, and likely to remain so. By the time they are old enough to question, they can't remember pulling out the baby teeth.

Ok, that's probably enough for now. If any of you have read this far, I'm very impressed! It's a lot to take in at one sitting, lol! Hopefully I have successfully managed to write it for everyone to understand - ie those who are not medically trained as well as those are. There are enough medically written articles on these subjects, but sadly not enough for those who don't understand the terminology. That makes a rare condition even for frustrating for most - because they simply cannot do their own research, because they cannot read what is written on the subject. I will be forever grateful that I was always interested in medicine, and have a medical background - it allowed me to be able to teach myself about this from the medical journals and articles published. I would never have gotten this far had I struggled to understand the basic terms used.