Congenital Insensitivity to Pain with Anhidrosis (CIPA)

So, Kellsey had her dermatology appointment today. It's really quite amazing. We got her referral, I called on Wednesday to schedule her appointment, and they got her in today. After waiting over a YEAR for an appointment in Nashville. Whatever!

Anyway, after talking for a bit about Kellsey's history, things she has been doing, even beyond the fact that she doesn't sweat (which is why we were there), and examining Kellsey, the dermatologist pulled out his medical book to show me an article about Congenital Insensitivity to Pain with Anhidrosis (CIPA). He said that Kellsey is a classic case. It matches up to Kellsey's symptoms and behaviors perfectly and answers a lot of questions about her.

CIPA is extremely rare... so rare in fact that this doctor has never actually seen a case in his office. He is sending Kellsey to a dermatologist in Denver to be seen for further testing and evaluation. He said we should get in in the next month or so.

SO, for those of you who want some light reading (haha) here are some articles on CIPA.

The Facts About Congenital Insensitivity to Pain with Anhidrosis

What is CIPA?

Congenital Insensitivity to Pain with Anhidrosis

For those of you who don't want to read and read, I'll give you the basic rundown...

CIPA is congenital which means it's inherited. We of course have no family history from her parents, so that's really neither here nor there.

Patients with CIPA feel no pain... we've known this about Kellsey for awhile. You might remember our problem when she first came home about her sucking on her fingers... She would suck on them until they were raw and bloody. She even sucked her fingernail off once. When I tried to get help for this, I was told it was her "soothing technique" and to let her be... even when her fingers were infected. @@ Thankfully we finally broke her of that habit, but lately she has taken to chewing on her lips at night... they are a horribly bloody mess. This helped confirm the diagnosis for her doctor today. I would post a picture, but really, it would just make you hurt. It makes ME hurt when I have to clean her up every morning... it doesn't make HER hurt though. She has fallen down our front steps in TN and cried because it scared her, but she never noticed that her leg was scraped and bleeding. She fell out the window of a playhouse at school last year and just stayed there, hanging half way out, head down, not making a sound, until her teacher noticed and went to get her. She had a huge bump on her head from hitting the concrete... which she never felt. And the list goes on.

Kids with CIPA have Anhidrosis which is the inability to sweat. Many times they die before age 3 due to overheating. Thankfully (??) Kellsey was in an orphanage where she wasn't outside all that often, and when she was, she was only in a diaper when it was warm out. When they ARE out in the heat their body temperatures shoot up high (which we know from experience with Kellsey) hers have gone as high as 105 within a matter of 20 minutes in the heat. This can lead to heat stroke and brain damage or death.

Eating for people with CIPA is a huge struggle... they don't feel hunger pain and many will mutilate their own mouths to the point where they have to end up having their teeth pulled (really praying we're not heading there now). Furthermore their taste buds are destroyed which makes eating... not enjoyable (??). Many of you know that eating has been a HUGE issue for us since she came home. We already knew she didn't taste, but this answers why. Any food preferences she has is purely a texture issue.

Also, people with CIPA cannot feel the urgency to urinate and therefore can only be toilet trained on a timed schedule... even then there is no guarantee there will not be accidents. They also have decreased GI motility and usually have constipation issues (we already have an appointment set up with GI for this next month... guess we have the answer now).

So... from here we go to dermatology in Denver where we will learn more about this condition and how to keep Kellsey safe. The dermatologist here is hoping to get her in within the next month. It's good to have answers... it explains a lot about Kellsey medically and behaviorally that we have been trying to get figured out since she came home. However, all these articles online are grim... most saying that individuals with CIPA do not live past the age of 25. That's a hard pill to swallow for your child. I'd like to wrap her in bubble wrap so she's sure to never injure herself... of course then she'd overheat! HA!

I realize I can't keep any of my children in a bubble (even though I REALLY try) and their lives are in God's hands. This will be another journey where we learn even more to put our trust in Him. As Kellsey nears her 5th birthday, I am more and more thankful she is here with us and not facing a transfer to an institution which would surely be a death sentence for her. *sigh*


I will of course update as I learn more at her future appointments.